Having a general check-up was also crucial for detecting hearing problems . There was a significant link between hearing problems and early adults who have isolated systolic hypertension. Hypertension awareness and having a general check-up had predictive value for detecting hearing problems in adults in the age range of 26~35 years. Therefore, public health strategies for hearing loss prevention might target this group by detecting and treating hypertension.Known as a silent disability, hearing loss is one of the major health burdens worldwide. Evidence implies that those suffering from hypertension can experience hearing disturbances. Self-reporting of hearing problems and self-reporting of hypertension may be useful in providing an alarm for detecting hearing problems. However, in the Indonesian population, this matter has not been properly reported. The aim of this study was to explore the prevalence of hearing problems and their relationships with other demographic factors. In total, 28,297 respondents of productive age from the Indonesian Family Life Survey 5th wave were assessed. A questionnaire and physical examination data were included in this survey. Self-reported hearing problems and their predictors were analyzed using univariate and multivariate logistic regressions. Hypertension awareness was a significant predictor of having a hearing problem (odds ratio (OR) [95% confidence interval (CI)],  The sense of hearing is important for humans to communicate with other people and also to interact within the community. With advancements in hearing technologies in the past few decades, identifying and diagnosing hearing impairment can be completed in many settings. Around 360 million people (over 5%) all over the world live with this disability (disabling hearing loss), which makes hearing loss a factor that is responsible for years lived with a disability ,2. AmongLoss of productivity and social isolation might result from communication difficulties caused by hearing impairment ,5. A sysBased on the National Health and Nutrition Examination Survey in 2015–2018, the prevalence of high blood pressure increases with age, at 28.2% among 20~44 year olds, at 60.1% in 45~64 year olds, and 77% among those older than 65 years . On the Epidemiological studies of hearing loss and hypertension as vascular risk factors supported the fact that there is a significant link between the two ,17. HoweIn this study, our main aim was to investigate the prevalence of hearing problems of various ages and blood pressure conditions. Later, we assessed the relationship between hearing problems and blood pressure based on subjects’ age groups in an Indonesian population.The Indonesian Family Life Survey 5th wave (IFLS5) 2014 was used in this study. The IFLS5 is a cross-sectional national survey that focuses on health and socioeconomic fields and was conducted from late 2014 to early 2015. Data were collected by assessing individual respondents, their households, and community levels. The IFLS survey is a longitudinal survey designed to provide public use data to investigate behaviors and outputs, and to the present, there have been five waves of surveys that are available on their website. In the first wave in 1993, 20 households from urban regions and 30 households from rural regions in 13 of 27 provinces in Indonesia were selected by random sampling. IFLS5 was chosen as the data source in this study because it has the most complete data compared to previous waves. A well-designed structured questionnaire was administered through in-person interviews by well-trained interviewers. A physical examination including blood pressure, body height, and body weight was also conducted . Our stuRespondents’ ages ranged from 15 to 64 years, which is considered productive age in the Indonesian population. Furthermore, age was divided into four groups based on Indonesia Ministry of Health criteria: (1) 15~25 years as adolescence, (2) 26~35 years old as early adulthood, (3) 36~45 years old as late adulthood, and (4) 46~64 years old as elderly.Blood pressure was measured three times by trained interviewers using an Omron meter (HEM-7203) on alternate arms in a seated position. A normal size cuff was generally used, while larger cuffs were also provided if needed. Averages of the systole and diastole data were determined. Later, blood pressure data were classified using the modified-American Heart Association (AHA) 2017 and Indonesian Society of Hypertension (INASH) 2019 criteria. AHA 2017  criteriaTo evaluate specific cases of ISH, additional criteria of the INASH 2019 with modifications were also added. The modified INASH version only has two groups which are non-ISH  and ISH. Originally, the INASH criteria had several groups of blood pressure, which included (1) optimal (systole < 120 mmHg and diastole < 80 mmHg), (2) normal (systole 120~129 mmHg and diastole 80~84 mmHg), (3) high-normal (systole 130~139 mmHg and diastole 85~89 mmHg), (4) grade 1 hypertension (systole 140~159 mmHg and diastole 90~99 mmHg), (5) grade 2 hypertension (systole 160~179 mmHg and diastole 100~109 mmHg), (6) grade 3 hypertension (systole ≥ 180 mmHg and diastole ≥ 110 mmHg), and (7) ISH (systole ≥ 140 mmHg and diastole < 90 mmHg) .Hearing problems were assessed by answering a question about hearing problems .Covariates consisted of hypertension-self reported, hypertension medication, body mass index (BMI), educational level, occupation, general check-ups, outpatient care, insurance coverage, and hearing aid usage. Information on hypertension self-reported was collected by answering a question about a diagnosis of hypertension (yes or no). Likewise, hypertension medication was reported by answering a question about a history of undergoing hypertension treatment (yes or no).2) from physical measurements. A Seca model 213 plastic height board was used to measure height to the closest millimeter. A Camry model EB1003 scale was used to calculate body weight to the nearest 0.1 kg. Finally, the BMI was categorized into four groups based on Indonesia Ministry of Health criteria, which are (1) underweight (BMI < 18.5 kg/m2), (2) normal (BMI 18.5~25 kg/m2), (3) overweight–mild (BMI 25.1~27 kg/m2), and (4) overweight–severe (BMI > 27 kg/m2).The BMI was calculated using height divided by weight squared  no schooling, (2) senior high school or lower (graduated), and (3) above senior high school (graduated). The occupational status was noted with questions about whether the respondent had worked within the past year and had the same job for more than 5 years. General check-ups, outpatient care, insurance, and hearing aid usage were recorded by answering questions of having had a general check-up in the last 5 years, having had outpatient care in the last 4 weeks, having a health card, and ever having to wear a hearing aid , respectively.t-test for continuous data were used. The odds ratio (OR), 95% confidence interval (CI), and p value were calculated using univariate and multivariate logistic regressions. A subgroup analysis was carried out by gender. All statistical analyses were performed with SPSS software vers. 26 . A p value of <0.05 was accepted as statistically significant.Descriptive analyses were used to show characteristics of study participants. The frequency, percentage, chi-squared test, or Fisher exact test for categorical data and the mean, standard deviation (SD), and Student’s Of the 28,297 total individuals (age (mean ± SD): 35.29 ± 12.78 years for men and 35.01 ± 12.79 years for women) from the Indonesian population, 220 people had hearing problems . The prevalence of self-reported hearing problems in those aged 15~25, 26~35, 36~45, and 46~64 years was 0.48, 0.48, 0.85, and 1.4 per 1000 persons, respectively. Thus, the prevalence of hearing problems increased with age. In addition, hearing problems were more prevalent among subjects with elevated blood pressure or hypertension, those with isolated hypertension, those taking antihypertensive drugs, those with self-reported hypertension, those employed, those with a general check-up in the past 5 years, and those with outpatient treatment in the last 4 weeks. Among those who were experiencing hearing problems, 51.4% were male and 48.6% were female, but none of them were using a hearing aid.p = 0.093). According to the criterion of the INASH 2019, the prevalence of isolated systolic hypertension was 6.3 per 100 persons.Overall, the hypertensive group comprised a majority of the population according to AHA 2017 criteria which was 12,843 participants. The prevalence of hypertension based on AHA 2017 criteria was 45.38 per 100 persons in this Indonesian population aged 15~64 years. Hypertension was not significantly correlated with hearing problems (p = 0.048) higher proportion of isolated hypertension (9.5%) than those without such problems (6.3%). Surprisingly, among those with hearing problems, only 26.8% were aware that they had hypertension and 7.7% were treating it with hypertension medication.Those participants with hearing problems had a significantly (p = 0.007), having had a recent general check-up (p < 0.001), and having had recent outpatient care (p = 0.003). Although the prevalence of hearing problems in the worker group at 77.3% was higher than in the non-worker group, the number of hearing problems was higher in the group who had had a general check-up in the past 5 years and those who had had outpatient care in the past 4 weeks.There were correlations of hearing problems with being employed  between hearing problems and age group, with the strongest protective effects in those aged 46–64 years. Otherwise, gender was not correlated with hearing problems (p = 0.144).p value; 1.373 [0.019~1.851], 0.037) and in the isolated hypertension group  compared to the reference group. This shows that having hearing problems was related to a hypertensive condition. Likewise, self-reported hypertension (3.127 [2.315~4.224] <0.001) and related treatment  had large risk effects on hearing problems.Based on all criteria for hypertension by AHA and INASH, significant risks were shown in the hypertension group  and outpatient care  among participants had significant risks for the occurrence of hearing problems. In addition, the underweight group based on the BMI had a significant protective effect against hearing problems  compared to the overweight group. Having a job seems to be related to hearing problems  compared to not having a job. Nevertheless, there was no relationship between hearing problems and educational level or insurance ownership .p < 0.05 of covariates  with hearing problems was next assessed. Subsequently, several models were developed to predict having hearing problems based on univariate variates . CompareFurther analysis showed that self-reported hypertension seemed to be an important predictor for having hearing problems as seen in model 1 . In addition, as seen in model 2, having a general check-up was a significant predictor for hearing problem conditions . These findings illustrate that hypertension awareness and having a general check-up are important factors; however, after adjusting for such factors, elevated blood pressure was still important to prevent hearing problems in early adults (26~35 years of age) .There was also a combined effect of age and blood pressure categories in INASH-isolated systolic hypertension which were shown in In addition, the subgroup analysis by gender had been performed. The result from the gender analysis showed insignificant results due to the small sample size in hearing problems .The study finding of a combined effect of age and blood pressure on hearing problems stresses the importance of the SBP condition in early adults (26~35 years of age) to prevent hearing problems. Hence, to eliminate the risk of hearing problems related to hypertension, public health professionals might target this population age range.There was a slightly higher prevalence of hearing problems in men (51.4%) than in women (48.6%) in this study. Meanwhile, the global prevalence of hearing loss at moderate or higher levels among men is slightly higher than that of women, at 217 million (5.6%) and 211 million (5.5%), respectively . Interesp < 0.05).Study findings in a Canadian population showed that the prevalence of hearing problems (79%) was higher among adults with hypertension . Additiop = 0.037) as well as the modified–isolated systolic hypertensive group (p = 0.05). This is similar to a previous study that found that hypertension was positively associated with hearing loss. To be exact, results of audiometric examinations showed that the hypertensive group had a higher hearing capacity (23.4 ± 8.67 dB) in comparison with the non-hypertensive group (18.3 ± 6.02 dB), and the longer a person had had hypertension, the worse was their hearing ability [p < 0.05) [Based on a modified AHA 2017 grouping of blood pressure, we also found that the hypertensive group was associated with hearing problems  . In 2019 < 0.05) . Additio < 0.05) .p < 0.001) [Supporting our finding that hearing loss was associated with both age and hypertension, there is also a study that indicated that hypertension was related to age (< 0.001) . In addi< 0.001) ,35,36. WAn increase in systolic blood pressure in early adults (26~35 years old) may be important, as it might increase the risk of hearing disturbances. So, we would encourage adults aged 26~35 years to have regular blood pressure check-ups and have early intervention if they have an elevated SBP in order to prevent hearing loss conditions. Moreover, this study emphasized the relationship between hearing problems and hypertension, and hence, this could be useful for future studies related to hypertension, especially in Indonesian populations.Apart from the subjectivity of self-reported hearing loss and self-reported hypertension, only a few indicators related to hearing disturbances were available in this study. This survey also did not have noise exposure information. In addition, there was a discrepancy between self-reported hypertension with blood pressure measurement results, and some of the blood pressure measurements were non-responded cases. Another limitation was the respondents’ volunteer effect, which might have been a factor that could have affected the results. We explored every variable which we thought to be useful in this study; however, these limitations were considerable since the survey mainly explored social aspects.There was a predictive value of self-reported hypertension (awareness) and having a general check-up against hearing problems in early adults. Thus, regularly monitoring the blood pressure can be suggested for this age group. Future studies are needed to investigate the mechanism of these findings.


Frontonasal dysplasia (FND) is a rare malformative complex affecting the frontal portion of the face, the eyes and the nose; it may occur singly or associated with other clinical signs. No systematic studies describing hearing in this condition were found.To evaluate hearing sensitivity and sound stimulus conduction from cochlea to brainstem in patients with clinical signs of FND.21 patients with isolated or syndromic FND were submitted to a clinical  and instrumental  hearing evaluation.A clinical, cross-sectional observational prospective study.Hearing thresholds were normal in 15 (70%) patients, abnormal in 5 (25%), mostly with conductive hearing loss; one patient did not cooperate with testing. The tympanometric curve was type A in 30 (72%) ears, type C in 5 (12%), type As in 4 (9%) and type B in 3 (7%). The auditory brainstem response (ABR) showed no abnormalities.Patients with FND showed no abnormalities in the auditory system from cochlea to brainstem in this study. Mild conductive hearing loss found in some is probably related to cleft palate. Further evaluation of hearing pathways at higher levels is recommended. Frontonasal dysplasia (FND) is a rare malformation characterized mainly by abnormalities in the eyes, nose, and frontal portion of the face. Clinical manifestations vary, and may include ocular hypertelorism, a wide nasal base and flat broad nose, a missing tip of the nose, a vertical cleft along the middle of the face affecting the nose and/or upper lip, a low-set frontal hairline , and an bifid anterior cranium occultumResearchers have reported several clinical findings in FND; involvement of the face may range from mild hypertelorism to a middle facial cleftWe found only case reports of FND with hearing loss in the Brazilian and international literature. Specific studies on hearing in FND were not present in the papers we compiled.Sensorineural hearing loss was reported in a female child aged 11 months diagnosed with FND and renal agenesisFour FND patients were studied, of which one had conductive hearing loss. This patient also presented cleft lip and palate, agenesis of the corpus callosum, delayed neuromotor development, and mental retardationA study has been published on a mother and daughter with craniofacial syndrome and limited abduction of the hips and shouldersConductive hearing loss was reported in a 4-year-old girl with FND and no CNS anomalies. Otoscopy revealed otitis media with effusion, and immittance testing showed a tympanometric curve without a compliance peak and bilateral absence of the stapedial reflexAnother case was a patient with FND, neuronal migration error, upper and lower limb lymphedema, and mild delayed neuropsychomotor developmentIf the phenotype of a given condition is known, anomalies that are not obvious but that are part of the condition should be investigated. The focus of most papers on FND has been the physical features of this disease; however, functions such as speech and hearing have not been explored in depth.Because of the importance of hearing for communications, and taking into account that individuals with craniofacial malformations are at risk for hearing, speech, and language disorders, it is relevant to check the function of the auditory system to diagnose possible conditions and reflect on treatment in these cases.Thus, the purpose of this study was to investigate auditory function in subjects with clinical signs of single or syndromic FND, and to measure the acuity of hearing and the integrity of pathways that conduct sound stimuli to the brainstem.The study was started following approval by the institutional review board; it met the requirements of Resolutions 196/96 and 251/97, and the approval protocol numbers were 368/2006 (4 December 2006) and 140/2010 (8 July 2010). All study subjects or their representatives signed a free informed consent form.The study sample comprised 21 subjects with a diagnosis of FND. The age of patients ranged from 7 to 42 years; the mean age was 19 years, the median was 16 years, and the mode was 8 years. There were 14 female subjects (66%) and seven male subjects (34%), totaling 21 subjects. Fourteen subjects (66%) were diagnosed with single FND and seven (34%) with syndromic FND; two of these (9.6%) were diagnosed with the craniofrontonasal syndrome, one (4.8%) with the cerebrofrontofacial syndrome, one (4.8%) with the oculoauriculofrontonasal syndrome, one (4.8%) with alar cleft and FND, one (4.8%) with agenesis of the corpus callosum, basal encephalocele, ocular anomalies and FND, and one (4.8%) with agenesis of the corpus callosum, encephalocele, mental retardation and FND. In 21 study subjects, seven (34%) had structural anomalies of the corpus callosum - four (19.2%) with agenesis, one (4.8%) with hypoplasia, one (4.8%) with a lipoma, and one (4.8%) with a pericallosal lipoma. Nine subjects (43%) had cleft palate that had already been treated surgically at the time of the evaluation, and 12 (57%) had normal palates.The audiologic evaluation consisted of five procedures: the clinical history, an ontological inspection , immittance testing, pure tone audiometry, and brainstem auditory evoked potentials (BAEP). A Hortmann Neuro-otometrie BERAmodul device was used for recording BAEP. The following parameters were used: 0.1 millisecond (ms) clicks, and 24 clicks presented per second. The analysis time was 10 ms, waves were recorded with the sum of 1,000 stimuli with alternated polarity; the study intensities were 80 dB when peripheral hearing was normal and the tracing was clearly visible; and were 90, 100 and 110 dB in cases of peripheral hearing loss or when tracings were altered at the initial 80 dB intensity; three recordings were made for each intensity. Stimuli were presented using Hortmann Neuro-Otometrie Beyerdynamic DT48 over-the-ear phones. During the test, patients were lying on a comfortable semi-reclined bed in an acoustic room.The electrophysiologic threshold was not measured, as the purpose of this study was to establish the integrity of auditory pathways, and not auditory sensitivity. BAEP tests were analyzed and compared with a normal reference. Normal parameters for analyzing latencies were the values obtained by biological calibration of the equipment.The audiometric results were normal in 15 subjects (70%) of 21; the audiometric results were abnormal in five subjects (25%), and one subject (5%) did not collaborate with testing. The findings were mild bilateral conduction hearing loss in two subjects (10%), mild unilateral conduction hearing loss in one subject (5%), mild unilateral isolated hearing loss at 6 and 8 kHz in one subject (5%), and mild unilateral isolated hearing loss at 8 kHz in one subject (5%).Forty ears were tested, of which 33 (82.5%) had normal audiometric thresholds and seven (17.5%) had altered audiometric thresholds.The results of tympanometry in 42 ears of 21 tested subjects were a type A curve in 30 ears (72%), a type C curve in five ears (12%), a type Ar curve in four ears (9%), and a type B curve in three ears (7%). Acoustic reflexes were present in 24 ears (57%), absent in 12 ears (29%), non-systematic in three ears (7%), and not measured in three ears (7%).BAEP findings were similar among the study sample; no altered neural conduction of auditory stimuli up to the brainstem was observed. The wave I, II, and III absolute latency values and the I-III, III-V, and I-V interpeak values were within normal limits.Previous studies have reported in detail the phenotype, and the variability of craniofacial and CNS malformations in FNDHearing has not been investigated in depth, and therefore there are no systematic data to compare with the present study. We found a 2:1 female to male ratio in our study sample, which differs from previously published dataThere is a known relationship between cleft palate and hearing loss - mostly conduction, but occasionally sensorineural hearing lossFND with cleft palate was present in about half of our study sample (43%); this finding is probably a peculiarity of our series, as our sample was taken from a center that specializes in the treatment of cleft lip and palate. All of these patients had already been treated surgically, and therefore had good velopharyngeal function.There are only five FND patients with hearing loss described in the literatureThe presence of anomalies in the skull, face, and CNS, as well as possible malformations affecting peripheral and central auditory pathways and/or other associated anomalies, which have been reported in most of these published cases, raise questions about possible etiologies for hearing loss, as only one of the FND cases in the literature had single FND without CNS anomalies. On the other hand, studies describing auditory anomalies in FND cases have been isolated case reports, which makes it difficult to infer whether the presence, type, and degree of hearing loss is part of a pattern or a non-systematic occurrence.In the present study of 21 FND cases, most of the subjects had normal hearing (76%); five subjects (24%) had hearing loss as demonstrated with audiometry, of which three (60%) had conduction hearing loss, and (40%) had sensorineural hearing loss. Of these five cases with hearing loss, two had single FND (40%) and three had syndromic FND (60%).A comparison showed that cleft palate was present in about 60% of hearing loss cases and in 30% of normal hearing subjects. A corpus callosum anomaly was found in 40% of patients with hearing loss, and in 30% of normal hearing subjects. These two anomalies were more frequent in patients with hearing loss - in particular cleft palate. Although hearing loss related with recurring otitis has been reported as being more frequent in patients with agenesis of the corpus callosum compared to normal individualsth cranial nerve to the superior olivary complex in the brainstem. Although conduction disorders classically increase absolute latencies in BAEP waves, this finding was not seen in our sample. It is possible that since these findings were mild in all patients, they may not have been sufficiently consistent to cause detectable delayed sound conduction by BAEP, of that this delay was not clinically meaningful since latency values were close to the maximum normal limit.Few of the study subjects had altered peripheral auditory pathways (the middle ear and cochlea). Neuronal conduction in the auditory pathways assessed by BAEP appeared to be intact from the distal portion of the 8Our series may be considered heterogeneous in terms of the genetic diagnoses, the clinical features, and age. However, the audiologic findings - which were similar among subjects - did not seem to reflect this heterogeneity.The present study underlines the possibility and importance of evaluating higher levels of the auditory system - including structures such as the cerebral cortex - with other electrophysiologic and behavioral tests, such as middle and long latency potentials and auditory processing tests. The normal results of the auditory system in our study may be considered as a starting point for this purpose. Molecular studies may be used in future studies to investigate genetic mutations, and may add much information because of recent advances in understanding the etiology of FNDThe basic audiologic evaluation - audiometry and immittance testing - revealed that auditory acuity was decreased in 33% of the study sample, consisting mostly of mild bilateral hearing loss. There were no auditory pathway disorders associated with sound stimulus conduction to the brainstem, as evaluated in the BAEP.FND and associated factors, such as CNS alterations, initially had no relation with hearing loss. The only factor that appears to be related with hearing loss is cleft palate, which may occur in isolation or associated with FND or other genetic conditions. It is a well-known etiological factor for conductive hearing loss.We concluded that patients with single or syndromic FND had no auditory alterations as part of their clinical manifestations, although these individuals are at risk of hearing loss. Further studies with more FND subjects and audiologic procedures are needed so that hearing profiles may be defined.


Case Report: The authors report a case of sudden deafness with unilateral tinnitus. The patients responded to therapy with Prednisone and Pentoxifylline after the diagnosis of acoustic neurinoma by imaging exams. Discussion: Sudden deafness can be described as an intense and abrupt sensorineural loss. Usually it is higher than 30 dB at three or more frequencies and develops in less than three days. Conclusion: Investigation of the etiology of sudden deafness is extremely important to establish the adequate strategy for the case.Vestibular schwannoma, also known as acoustic neurinoma, is the most frequent tumor of the cerebellopontine angle, and represents 9% of all intracranial tumors.  Sudden hearing loss may be a case of abrupt and intense sensorineural hearing loss. It is usually above 30 dB, involving three or more contiguous frequencies, and sets-in in less than three daysThe causes of sudden hearing loss frequently raise doubts and controversies as to etiology, evolution and treatment. Virus is the predominant etiology, and it may have vascular, autoimmune, thyroid glad, LUES, labyrinthine fistulas, ototoxicity, trauma, neuropathies and schwannomas as possible causesWe know that tumors of the cerebellopontine angle, such as vestibular schwannomas may cause sudden hearing loss.Vestibular schwannomas, also known as acoustic neuromas, are the most frequent type of tumor of the pontine angle, corresponding to approximately 9% of all intracranial tumors. It is a benign tumor that springs from the Schwann cells, more frequently in the upper branch of the vestibular nerve. It grows slowly towards the pontine angle, pushing against the VIII cranial nerve and enlarging the internal acoustic meatusMost of the times, initial symptoms are neurotological: progressive unilateral sensorineural hearing loss, tinnitus and vertigo. When it reaches bigger sizes, it may not only affect the facial and the trigeminal nerves , but also present central neurological manifestations. The labyrinthine alteration does not happen in a crisis fashion - differentiating it from Meniere’s diseaseThe sudden unilateral hearing loss caused by the tumor is the major symptom, happening in up to 26% of the acoustic nerve tumors. A spasm or occlusion of the labyrinthine artery as the tumor pushes against it may be the reason for hearing loss.In diagnosis, besides a detailed history and physical exam, it is necessary to run a complete audiologic evaluation with vestibular tests, to assess the trigeminal nerve, and to do a gadolinium contrasted MRITreatment of choice is surgical removal, and the access approach will depend on its location and extension.The authors report a case of recurrent sudden hearing loss and tinnitus caused by a vestibular schwannoma.W. A. O., 38 years old, caucasian, married, repair shop manager, born in Bauru/ SP. Went to an otorhinolaryngologist for the first time in January of 2004, complaining of sudden left ear hearing loss without any triggering factors, lasting for two days already. He also complained of continuous high-pitch tinnitus in the same ear. He had no vertigo or neurological alterations.He did not report prior pathologies  and use of medications.He was normal under physical examination, with intact and shiny bilateral tympanic membranes, mild nasal septum deviation to the right side, tonsils grade I, no palpable lymph nodes.His audiogram showed severe sensorineural hearing loss after 3000Hz, with SRI of 92% and SRT of 15 dB in his left ear; his right ear was within normal limits.Based on this we suspected of sudden hearing loss and ordered a blood work up. The patient was started on Prednisone 60 mg/day and Pentoxifylline 800mg/day.After one week the patient returned saying he had a partial improvement in both hearing and tinnitus.Lab exams: CBC normal; fasting glucose 103 mg/dL, total cholesterol 175 mg/dL, triglycerides 481 mg/dL, VDRL negative.A new audiogram did not show alterations in his right ear and showed a light sensorineural hearing loss after 3000 Hz in his left ear, SRI 100%.The patient was educated as to a better diet and medicated with Gingko biloba 240mg/day. We ordered an MRI and BERA.The patient returned five months later, with a new manifestation of hearing loss and a worsening in his tinnitus in the left ear, with four days of evolution and he did not have vertigo.Audiogram showed severe sensorineural hearing loss in the high frequencies in this left ear - similar to the first one. He was once again medicated with prednisone 60 mg/day and Pentoxifylline 800 mg/day, and reported a little improvement after he started with the medication.Another MRI was ordered, this time showing an expansive solid mass, measuring 5mm in diameter, inside the internal acoustic meatus (vestibular schwannoma).The patient was referred to a neurosurgeon, who decided to observe him clinically.Sudden hearing loss may be described as an abrupt and intense sensorineural hearing loss. It is usually above 30 dB, in three or more contiguous frequencies and takes less than three days to develop. Patients know exactly when it started, it is usually unilateral and may be permanent or not.The causes of sudden hearing loss frequently bring doubts and controversies as to its etiology, evolution and treatment. It is known that tumors of the pontine angle, such as vestibular schwannomas may cause sudden hearing loss. Symptoms onset and duration are not strictly related to tumor sizeHearing loss in cases of vestibular schwannoma is usually unilateral and progressive, however it may happen suddenly and involve hearing discrimination more than auditory acuity.Sudden hearing loss usually occurs thanks to an occlusion of the labyrinthine artery. It is yet unknown whether this sudden occlusion is caused by extrinsic compression alone or if there is any intravascular involvement (thrombosis), or a problem on the vessel wall , or some neuroendocrine factor affecting the artery (vasoconstriction)We presented a case in which sudden hearing loss and tinnitus were the only symptoms the patient had, for whom we initially ordered audiometric exams, followed by laboratory exams and the patient was put on Prednisone and PentoxifilinIt is highly important to investigate the etiology in cases of sudden hearing loss, in order to better manage and treat such a patient. The otorhinolaryngologist must have in mind all the possible etiologies of sudden hearing loss and the complementary tests available for diagnosis.


We describe the macular morpho-functional assessment of a 65-year-old man affected by stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR), studied by visual field, SD-OCT, autofluorescence, full-field electroretinogram (ffERG), multifocal electroretinogram (mfERG) and multifocal Photopic Negative Response (mfPhNR) recordings. The typical presentation consists of the foveal appearance of radial cartwheel pattern for the splitting of the retinal layers at the level of the Henle fiber layer (HFL) and the outer plexiform layer (OPL), perfectly seen by Spectral Domain-Optical Coherence Tomography (SD-OCT). Despite a normal function of the outer retina of the peripheral and central retina evaluated by ffERG and mfERG respectively, we observed a reduced function of the retinal elements involved in the retinoschisis by recording mfPhNR that assesses mainly inner retina function . Therefore, it is likely that the observed impaired mfPhNR responses reflect the signaling defects derived from the delaminated middle retina and transmitted to the innermost retinal layers. Stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR), firstly described by Ober et al. , is an uElectro-functional studies were also performed on our patient. Dark- and light-adapted full-field electroretinograms (ffERG), 10 Hz and 30 Hz flicker ERGs  have been recorded according to the International Society for Clinical Electrophysiology of Vision (ISCEV) standards ,7. NormaIn this case of SNIFR, we were able to establish the typical appearance of inner retina delamination by SD-OCT, as well as slight signs of morphological outer retina changes in the fovea . These findings, however, were not associated to an impairment of the peripheral nor the central outer retina function. Indeed, dark- and light-adapted ffERG as well as 10 Hz and 30 Hz flicker ERGs recorded normal a- and b-wave amplitudes, and normal photoreceptors and bipolar cells function by mfERG was recorded from localized retinal areas of the macula. Our functional findings are in contrast with those reported by Yadav et al. , who desAlthough accurate multimodal imaging of retinas has been already performed by previous authors in SNIFR ,2,3,4,5,Therefore, by recording mfPhNR in our case of SNIFR we found reduced RADs in all rings, likely reflecting the signaling defects arising from the delaminated middle retina to the innermost retinal layers, passing through the transmission of the intraretinal connection cells . The application of this electrofunctional method allowed us to identify, for the first time, in this rare condition of SNIFR, the overall dysfunction of macular retinal ganglion cells along with their axons. This aspect, associated with an absence of outer retinal dysfunction , could help us to make a differential diagnosis between SNIFR and other hereditary macular schisis ,15. The


Age-related hearing loss is a widespread condition among the elderly, affecting communication and social participation. Given its high incidence, it is not unusual that individuals suffering from age-related hearing loss also suffer from other age-related neurodegenerative diseases, a scenario which severely impacts their quality of life. Furthermore, recent studies have identified hearing loss as a relevant risk factor for the development of dementia due to Alzheimer’s disease, although the underlying associations are still unclear. In order to cope with the continuous flow of auditory information, the brain needs to separate repetitive sounds from rare, unexpected sounds, which may be relevant. This process, known as deviance detection, is a key component of the sensory perception theory of predictive coding. According to this framework, the brain would use the available incoming information to make predictions about the environment and signal the unexpected stimuli that break those predictions. Such a system can be easily impaired by the distortion of auditory information processing that accompanies hearing loss. Changes in cholinergic neuromodulation have been found to alter auditory deviance detection both in humans and animal models. Interestingly, some theories propose a role for acetylcholine in the development of Alzheimer’s disease, the most common type of dementia. Acetylcholine is involved in multiple neurobiological processes such as attention, learning, memory, arousal, sleep and/or cognitive reinforcement, and has direct influence on the auditory system at the levels of the inferior colliculus and auditory cortex. Here we comment on the possible links between acetylcholine, hearing loss, and Alzheimer’s disease, and association that is worth further investigation. Age-related hearing loss is the most prevalent disability/disease-related condition in the world . DisabliEven moderate age-related hearing loss causes deficits of speech understanding in many otherwise cognitively intact, aging people, and subsequently, many of these individuals withdraw from active participation in society. Moreover, this problem is epidemiologically associated with cognitive impairments that typically affect the elderly, such as dementia due to Alzheimer’s disease (AD), to the point that there may exist a causal relationship even when ruling out the effect of age. This scenario profoundly influences the quality of life of these individuals and their families and causes a substantial impact on social welfare and health system costs. Given the lack of established disease-modifying treatments for any neurodegenerative cause of dementia, a better knowledge of the mechanisms of the alterations that occur in these neurodegenerative pathologies is necessary to prevent their occurrence, and thus it has become a world priority. Indeed, in a seminal work launched in 2017, the Lancet’s “Commission on dementia prevention, intervention, and care”  proposedThe links between hearing loss and dementia are incompletely elucidated and are under debate . DespiteIn a recent detailed study, There are many different types of dementia, and AD is the most common, followed by vascular dementia and dementia with Lewy bodies. Mixed dementia with features of more than one cause is also common, as well as frontotemporal degeneration and dementias associated with brain injury, infections, and alcohol abuse, although less widespread. Cognitive deficits in neurodegenerative diseases have often been characterized as the loss of core functional modules in distinct brain regions, such as “memory centers” or “executive centers.” This classical approach emphasizes the functional difference between disorders, at a time when preclinical models suggest convergence in the pathophysiology of different diseases . Furtherdeviance detection.Sound plays an essential role in human life, starting from basic processes of survival  to many human activities . Therefore, the ability to hear and properly recognize sounds is critical to human perception. Humans, like other animals, live with the constant and often overwhelming flow of sensory information coming from the environment. For perception of behaviorally relevant stimuli to occur, sensory systems are organized to identify information sources efficiently. This is true in the auditory system, which is highly complex anatomically and physiologically , 2015. Ipredictive coding theory  of the synapses of cholinergic neurons in the hippocampus or nucleus basalis of Meynert . There iACh is a widely distributed neuromodulator throughout the brain, including the inferior colliculus (IC) and the auditory cortex (AC). Neuromodulation by ACh has been found to have a direct effect on the deviance detection processing carried out by neurons located in the IC, a midbrain auditory station where almost all ascending auditory information converges before progressing onto the auditory thalamus and, ultimately, the AC. The IC is the first stage in the ascending auditory pathway to show stimulus-specific adaptation (SSA), a neuronal phenomenon proposed to contribute to the generation of deviance detection at a cellular level . SSA in In the AC, ACh modulates different neurobiological processes such as attention, learning, memory, arousal, sleep and/or cognitive reinforcement . The maiNeuromodulatory inputs such as ACh can shift the relative contribution of bottom-up and top-down signals influencing prediction errors. But how different levels of modulatory inputs alter the sensitivity by which brain circuits prioritize and respond to sensory information or report prediction errors remains unclear. AD is characterized by declines in brain cholinergic neurons e.g., , and cogIn conclusion, there is increasing evidence in the literature of links between Alzheimer’s disease, hearing loss and deviance detection. How are they related to each other is a topic that deserves to be further explored, and potentially could shed light on some basic features of brain function, as well as on the consequences suffered by AD patients. Taken together, the studies mentioned in this Perspective suggest that a core cognitive deficit in AD may involve a deficiency of predictive coding, and this deficiency may be related to the loss of cholinergic adjustment of weighting of bottom-up and top-down inputs in conjunction with loss of auditory sensory input. To better understand the relationship between hearing loss and AD, future studies should examine whether modulation of peripheral hearing and/or cholinergic tone alters auditory predictive behavior or cognitive function more generally. While the alterations related to the cholinergic system seem to play common roles in both hearing loss and AD pathogeny, other intricate mechanisms could also be involved. Amyloid beta toxicity, oxidative stress, and chronic inflammation could be also relevant for explaining this association between two pathologies with significant impact in humans and are worth to be studied in the future.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author/s.DP-G, TS, DL, and MM wrote the manuscript. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. 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Hearing loss has been associated with serious health problems around the globe. Previous studies have found the positive effects of fitting hearing aids on health, but few studies were conducted in developing countries. The aim of this study is to examine the effects of hearing aids on multiple health outcomes among middle-aged and older adults with hearing loss in rural China.In this randomized controlled trial (RCT), participants aged 45 and above were randomly assigned to the treatment group prescribing with hearing aids or to the control group with no intervention. Trial outcomes for 385 participants were collected during the 20-month follow-up study. Using the difference-in-difference approach, our primary outcomes were hearing-related quality of life (QoL) and generic QoL.p = 0.005), HHIE-S-Emotional scores , and HHIE-S-Situational scores . The intervention was also effective in alleviating the increase in depressive symptoms . Subgroup analysis revealed that the effects were only shown among people with social activities or active social networks.The intervention led to improvements in hearing-related QoL, presenting as the reduction in Hearing Handicap Inventory for the Elderly Screening Version (HHIE-S) scores (interaction coefficient = − 2.86, Our study is the first RCT in China to measure the health effects and heterogeneity of hearing aid interventions. Wearing hearing aids can help improve hearing-related QoL and alleviate the increase in depressive symptoms. The intervention is expected to be applicable to similar settings in other developing countries to combat hearing-related health problems.ChiCTR1900024739. Registered on 26 July 2019.Chinese Clinical Trial Registry,  Hearing loss is the most common sensory dysfunction in human beings and is also a common health issue around the globe . The WorHearing loss affects people’s quality of life (QoL) widely . HearingHearing aid use is currently the most common intervention for age-related hearing loss in adults . ResearcThe results on the effects of hearing aids on broader health outcomes such as mental health, physical health, cognitive function, and social engagement are also inconsistent to some degree. On the one hand, studies found that those using hearing aids had no significant improvement in mental health, cognitive function, social participation, or quality of life compared with the non-users –23. On tDue to the concealed and progressive nature of age-related hearing loss, individuals especially older adults do not much pay attention to it. Besides, the high costs of hearing aids hinder their use . A systeBased on previous literature, we hypothesized that (1) the hearing aid intervention led to improvements in hearing-related QoL and generic QoL; (2) the intervention reduced the probability of having chronic diseases, impaired ADLs or IADLs, and depressive symptoms; and (3) the effects were different between people with and without social activities or active social network. Our study is novel in that it focuses on a rural sample, while previous studies are not confined to rural communities. Secondly, it includes a diverse set of assessments including evaluations of hearing, depressive symptoms, QoL, ADL/IADL, and social functioning, as existing studies often do not account for all these various factors. In addition, we performed subgroup analysis by social activities and social networks to look at the effect modification, which has never been done before. Our work is of value to a broad audience as it provides the latest evidence in areas of medical and health advances for promoting hearing rehabilitation and achieving healthy aging in developing countries.Our trial is a two-arm, randomized controlled trial (RCT) conducted in Linyi City, Shandong Province, China. It aims to improve the QoL of middle-aged and older adults with hearing loss by providing free hearing aids to them. Audiometric testing of pure-tone audiometry (PTA) at the thresholds of 0.5, 1, 2, and 4 kHz was used to assess the degree of hearing loss. PTA was assessed by the study audiologist in an independent audiometry room (background noise ≤ 40 dB) by presenting a pure tone to the ear through an earphone and measuring the lowest intensity in dB at which this tone is perceived 50% of the time.The inclusion criteria are (1) aged 45 or above, (2) clinically diagnosed with moderate or above hearing loss (defined as a PTA of the four frequencies > 40 dB in the better hearing ear ), (3) noOf the total sample, 29.61% had a moderate hearing loss, 37.40% had a severe hearing loss, and 32.99% had a profound hearing loss  is adopted to measure hearing-related QoL. HHIE-S has 10 questions, including 5 emotional questions such as “Do you feel handicapped by a hearing problem?” and 5 situational questions such as “Does a hearing problem cause you difficulty when attending a party?” Subjects are required to answer “yes,” “sometimes,” or “no” according to their own real situation, and the corresponding scores were 4, 2, and 0, respectively. The total score is 0–40 points. A higher score denotes worse hearing-related QoL. A total score of more than 8 is generally considered to represent having hearing loss. The Chinese version of HHIE-S has been proved to be feasible, reliable, and of validity in the measurement of hearing-related QoL in China .Generic QoL: a. The 12-item Short-Form Health Survey (SF-12) is used to summarize generic QoL. One of the questions is “Does your health now limit you in climbing several flights of stairs?” It describes QoL from eight dimensions, including physical function, role limitation caused by physical problems, body pain, physical health awareness, social function, role limitation caused by emotional problems, emotional health, and mental health awareness . Among tGeneric QoL: b. The EuroQoL 5-Dimension 5-Level (EQ-5D-5L) is adopted to measure generic QoL in terms of mobility, self-care, daily living, pain/discomfort, and anxiety/depression . For exaPrimary outcomes are hearing-related QoL and generic QoL. We measured all outcomes at baseline and 20-month follow-up.Chronic diseases: Subjects are asked whether they have at least one chronic disease that has been diagnosed by a doctor, including hypertension, dyslipidemia, diabetes, cancer, chronic lung diseases, liver diseases, heart diseases, stroke, kidney diseases, stomach diseases, digestive system diseases, mental health problems, diseases associated with memory , arthritis, rheumatism, or asthma.Activities of daily living (ADLs): ADLs are used to measure subjects’ physical functioning. Impaired ADLs are defined as the subject having difficulty or inability to perform any of the activities of daily living, including dressing, bathing, eating, getting up or out of bed, going to the toilet, or controlling urine and feces .Instrumental Activities of Daily Living (IADLs): IADLs are defined as whether the subject is having difficulty or inability to perform any of the instrumental activities of daily living, including doing housework, cooking, shopping, making phone calls, taking medications, or managing finances .Depressive symptoms: Depressive symptoms of the subjects are detected by using the 10-Item Center for the Epidemiological Studies of Depression Short Form (CES-D-10) . The subCognitive function: The subjects’ mental state and cognitive function deficit are tested by the Mini-Mental State Examination (MMSE) . The scaSecondary outcomes include chronic diseases, activities of daily living (ADLs), instrumental activities of daily living (IADLs), depressive symptoms, and cognitive function. We measured all outcomes at baseline and 20-month follow-up.Subjects were asked whether they participated in one or more social activities last month, such as socializing with friends; playing mahjong, chess, or cards; going to the community room; offering to help a relative, friend, or neighbor who did not live together; dancing, exercising, and playing Qigong; joining in activities organized by associations; participating in volunteer activities or charitable activities; caring for the sick or disabled who did not live together; attending school or training programs; speculating in stocks, funds, or other financial securities; and surfing the Internet.The Lubben Social Network Scale (LSNS) is a brief instrument used to evaluate the size, closeness, and frequency of contacts of a subject’s perceived social network  . The scaT-test for continuous variables and Pearson’s chi-square test for categorical variables were used to examine the extent to which there was a balance between characteristics of the intervention and control groups at baseline. Difference-in-difference (DID) analysis was used to analyze the effect of the intervention. To be exact, regression models containing time dummy (the 20-month follow-up and baseline [reference]), randomization group (intervention and control [reference]), and a time × group interaction were used to estimate the treatment effects, which allowed us to understand the impact of treatment on outcomes from baseline to the end of the follow-up period [p period . The regYit is the outcome variable for an individual i in the period t; INTERVENTIONi is a dummy variable, which takes the value of 1 if the individual received intervention; otherwise, it is 0; PERIODt is the dummy variable for the period, which is 1 if the intervention is implemented, and 0 if not; the interaction term of INTERVENTIONi × PERIODt measures the effectiveness of the intervention, and β3 is the true effect of the intervention. Xit is a set of control variables, including the individual’s age, sex, household registration types, education levels, marital status, job types, life sources, medical insurance, pure-tone average, smoking status, drinking status, social activities, and social network.where Since social activities and social networks may affect the impact of the hearing rehabilitation intervention, we performed subgroup analysis by social activities and social networks to look at the effect modification. The analysis using all data was performed on the basis of “intention-to-treat (ITT).” We present the covariate-adjusted results as our main results because of the potential gains in power and precision. Stata version 16  was used for all analyses.Table p<0.001), indicating that those in the treatment group had worse hearing-related QoL. The other primary and secondary outcomes did not show significant differences between the treatment group and the control group. Overall, the characteristics of samples from baseline and follow-up surveys were well balanced between the groups.Nearly 70% of the sample had severe or profound hearing loss. More than half of the sample never smoked (51.43%) or drank alcohol (52.47%). 74.03% of the sample had engaged in at least one social activity in the past month, and 73.77% of the sample had an active social network. Age, sex, hukou types, education, work type, life sources, health insurance, pure-tone average, smoking status, drinking status, social activities, and social network showed no significant differences between the treatment and control groups. The HHIE-S scores were significantly higher for those in the treatment group than those in the control group at baseline  for the baseline sample and 17.40 (SD = 0.46) for the follow-up sample, as most of the respondents were poorly educated (illiterate or just finishing primary school) and had great difficulty hearing.p = 0.005), hearing-related emotional QoL , and hearing-related situational QoL . For secondary outcomes, the intervention significantly alleviated the increase in depressive symptoms .Table p = 0.001), hearing-related emotional QoL , and hearing-related situational QoL . For individuals who had active social network, the hearing aid intervention significantly improved hearing-related QoL  and hearing-related situational QoL  and significantly alleviated the increase in depressive symptoms .Table Our RCT is one of the first rigorously designed trials on the effects of providing free hearing aids on multiple health outcomes in developing countries. The results showed that the intervention was to some extent effective in improving hearing-related QoL but had no effects on generic QoL. The intervention also alleviated the increase in depressive symptoms in the 20-month follow-up for middle-aged and older adults in rural China. The subgroup analysis revealed that the effects were only shown among people with social activities or active social networks. That is, the intervention was effective for those who were socially active. As most previous trials that have attempted to improve health by wearing hearing aids have been done in high-income countries, our study provides a promising vision of hearing aid fitting strategies for developing countries.Although the results were statistically significant, the HHIE-S score of the treatment group only improved a little from baseline to follow-up. However, we should also account for the fact that the HHIE-S score of the control group worsened from baseline to follow-up, making the total effect size larger. In addition, it appears that participants in the treatment arm actually had more depressive symptoms at follow-up than they did at baseline, which was possibly due to older age or the COVID-19. The intervention actually alleviated the increase in depressive symptoms in that the increase in depressive symptoms was not as large in the treatment group as in the control group. Our results were consistent with existing studies. For example, one pioneering study found that, compared with those in the control group, the social emotional function and communication ability measured by the HHIE of older adults with hearing loss were significantly improved, and their depressive symptoms were significantly reduced after wearing hearing aids .However, there were no effects of the hearing aid intervention on generic QoL, which also conformed to the previous literature. For example, Jorre et al. found no significant improvements in mobility, self-care, daily activities, and pain/discomfort in the five dimensions of QoL measured by EQ-5D after wearing hearing aids , 18. StaHeterogeneity analysis found that the effects of the hearing aid intervention on health outcomes varied by social activities and social networks across people. For adults with no social activities or with restricted social networks, the hearing aid intervention did not improve their health. For adults participating in at least one social activity, the hearing aid intervention significantly improved hearing-related QoL and its emotional and situational dimension. For adults with active social networks, the hearing aid intervention not only improved hearing-related QoL, but also significantly alleviated the increase in depressive symptoms. Hearing loss has been thought to be associated with reduced social interaction and emotional communication . It can Our study is the first RCT in China to measure the effects of the hearing aid intervention and its heterogeneity in hearing-impaired middle-aged and older adults. Strengths of the study include its randomized controlled design, a large sample size, and high (> 85%) rates of follow-up, which tend to increase confidence in the significance of the results. However, there are also several limitations. First, as the follow-up data were collected during the COVID-19 pandemic, the related restrictions on socialization and mental well-being may potentially reduce the effect sizes. Second, given that most individuals had severe to profound hearing loss and cognitive impairment, we should be cautious when generalizing the results to a larger population. Fourth, our RCT with an open hearing aid is a limitation, as expectancy-based placebo effects can affect the outcomes prone to expectancy results, such as the self-reported hearing-related measure and the depression measure. Future studies could take into account other factors like the pandemic or hearing aid compliance and generalize the results to a broader population.To conclude, our study proves that proving free hearing aids substantially improved hearing-related QoL and alleviated the increase in depressive symptoms for middle-aged and older adults with hearing loss in rural China. It is imperative that middle and older adults with age-related hearing loss wear hearing aids regularly to recover their hearing, as well as strengthen their social participation and social network to further improve QoL. The intervention is expected to be scaled up through its integration within the health insurance system in China and be adapted to other similar settings in developing countries facing the problem of untreated hearing loss.


Conscripts are exposed to various sources of impulse noise despite hearing protection recommendations. The aim of this study was to investigate the frequency of acute acoustic trauma (AAT) among conscripts after exposure to assault rifle noise in the Finnish Defence Forces (FDF). This nationwide population-based cohort comprised all conscripts  in the FDF during the years 1997–2003 and 2008–2010. We included those who claimed to have AAT symptoms from assault rifle noise during the study periods. During the investigated 10 years, 1617 conscripts  experienced a new hearing loss due to AAT. Altogether, 1456 (90%) of all AAT-induced hearing losses were caused by rifle-caliber weapons and 1304 (90%) of them when firing a blank cartridge. There was no clear diminishing trend in the annual numbers of AATs. In 1277 (88%) incidents, no hearing protector was used. Tinnitus was the most prominent symptom. Hearing losses after AAT were typically mild, but serious deficits also occurred. In conclusion, we found that 0.7–1.5% of the conscripts experienced an AAT during their service in the FDF. Most incidents occurred when firing a blank cartridge with a rifle-caliber weapon and with no hearing protector in use. Finland has a general law of conscription. Military (or non-military) service is obligatory for every Finnish male citizen between 18 to 60 years of age. Voluntary military service was made possible for women in 1995. Conscript service is usually carried out at the age of 19 years, and it lasts for 165, 255 or 347 days. During the service, a conscript may be exposed to impulse noise from various firearms as the shooting conditions vary both in the field circumstances and at a shooting range [There are many possibilities for the exposure to impulse-noise in military environment, including various small arms . MeasureThe hearing protection guidelines in the FDF date back to 1968, when hearing protectors (ear plugs) were instructed to be used at shooting ranges. Earmuffs became mandatory a decade later (FDF 1979) for sheltered shooting-range conditions. However, the use of ear plugs only was allowed when shooting at battle shooting situations or at open shooting ranges. According to these regulations, the used hearing protection may not risk general safety or cause undue harm in military operations. It remains obvious that these limitations and the discomfort of various types of ear plugs led to insufficient hearing protection among soldiers. Therefore, the supplementary and revised regulations (FDF 1985) ordered the use of at least earplug protectors for all shooting situations. Further, later regulations  gave instructions regarding protector types and safety distances for persons responsible for military education and training.A strong acoustic trauma will induce histological changes in the cochlea and auditory nerve. Cochlear outer hair cell loss and damage of inner hair cell stereocilia and axonal loss and myelin sheath disorganization of the auditory nerve will consequently lead to permanent hearing loss .Typically, hearing loss after acute acoustic trauma (AAT) can first be seen at 6 kHz frequency, and then at 8 kHz and 4 kHz ,11. HighThere are no reports on the occurrence of AAT in the FDF since the study by Mrena et al. (2004) including a selective series of 163 AAT patients treated at the Central Military Hospital during the year 2000 . Most ofThe primary aim of the present study was to investigate the population-based occurrence of AATs after exposure to assault rifle noise during conscript training in the FDF. The secondary aim was to evaluate the possible causes for such accidents.In the FDF a conscript is instructed to immediately report a suspected AAT and fill in a questionnaire , which sThis retrospective nationwide population-based study consists of two cohorts covering the years 1997–2003 and 2008–2010. Information on 2004–2007 does not exist, based on organizational causes. The large population consists of all FDF conscripts in service during these two time periods . The inclusion criterium was that if the conscript complained of changed hearing after exposure to assault rifle noise, then a questionnaire was filled in and later an audiometric analysis was performed. All conscripts who had filled in the questionnaire were thus included in this study. It was still possible that the audiometric analysis later showed only a rather limited hearing threshold shift. The study cohort comprised all conscripts (1456) during the same time periods who claimed to have symptoms of an AAT, i.e., ear pain, dullness, tinnitus or a subjective hearing deficit after exposure to assault rifle noise. These subjective symptoms, combined with a hearing threshold change of at least 10 dB for one frequency in the speech area (frequencies 500 Hz–4 kHz) or a change of at least 15 dB for the other frequencies, compared with a prior audiogram, were considered an AAT-induced hearing deficit.The subjects were asked to fill in a questionnaire including the following parameters: type of weapon and cartridge, user of the weapon (own weapon or belonging to another shooter), distance from the weapon, type of hearing protection used, reason for not wearing hearing protection, prior instruction on the use of hearing protection. A written description of the incident was also obtained . These rAll Finnish conscripts are screened for their hearing at the beginning of their military service. In this screening examination, a 20 dB hearing level is measured at frequencies of 0.5, 1, 2, 3, 4, 6 and 8 kHz in a standardized auditory booth. If the hearing threshold is higher than 20 dB, then the hearing threshold of this frequency is examined more carefully. After a noise incident, accurate hearing thresholds are always examined.In cases of an AAT-induced hearing loss, it is compulsory to consult an ENT (ear-nose-throat) specialist for evaluation of further care. If the AAT-related deficit exceeds 10 dB at 0.5–2 kHz or more than 35 dB at 3–8 kHz, then the conscript is ordered to receive hyperbaric oxygen treatment. Currently, corticosteroids and hyperbaric oxygen therapy are the standard treatment of acoustic trauma in the FDF. However, during the study periods, normobaric or hyperbaric oxygen only was used. Milder deficits are monitored with a control audiogram after two weeks. In addition, the conscript is ordered not to attend noise-causing service for a certain period of time.Sound pressure levels (SPLs) of weapons can be measured by using sound level meters, hearing loss dB is measured by using audiometers, and attenuation of hearing protectors by using an insertion loss factor in field conditions with sound-level meters (microphone outside and inside hearing protector). However, the information data on hearing protector attenuation values are measured in laboratory conditions by using the human threshold of hearing (dB).The study proposal received approval from the Research Ethics Committee of the Helsinki and Uusimaa Hospital District Occupational Health Ethics Committee (Dnro 315/E2/03/27.6.2003) and from the Coordinating Ethics Committee of the Helsinki and Uusimaa Hospital District (Dnro 41/13/03/00/2012). Study permissions were granted by FDF on 7 May 2003 (Dnro R680/8/E/II) and 29 March 2012 (Dnro 2148/26/2012/AI6100).During the investigated periods  the total number of conscripts with a new hearing loss due to AAT in the FDF was 1617 . There were 161 AATs after exposure to noise from large-caliber weapons and explosions. Altogether, 1456 (90%) of all hearing losses were caused by rifle-caliber weapons. This is around 0.7–1.5% of the annual number of conscripts . Altogether, 1150 rifle-caliber AATs were registered during the first study period  and 306 during the second period . The audiograms of Group I were measured on the same day of the incident in 73%, on the following day in 15%, later in 3%, and the timepoint was unknown in 9%. The audiograms of Group II were measured during the same day in 44%, the following day in 17 %, later in 4%, and on an unknown day in 35%. There were 1439 (99%) males in the study cohort and 17 females (1%).There were 841 (73% of the Group I) unexpected shots in the Group I and 230 (75%) in the Group II. Normal cartridges caused 16 (1%) AATs in the Group I and 5 (2%) AATs in the Group II.The annual numbers of AATs from assault rifle-caliber weapons in the FDF showed a slightly diminishing trend during the study periods .Rifle-caliber weapons consisted of assault rifles, pistols and light machine guns. Acute acoustic traumas were mostly noted in February and November, which relates to the first shooting practices that the conscripts perform in their service. Most hearing losses were due to firing an assault rifle with a blank cartridge during military drills . When using normal cartridges, there was a recorded AAT-related hearing loss information only in 1% of the cases, typically at shooting ranges. Typically, in 593 (41%) out of the 1456 cases the neighboring conscript was shooting. Among the whole cohort of 1456 persons, the distance was less than 2 m for 973 (67%), 2–5 m for 257 (18%), more than 5 m for 84 (6%), and unknown for 142 (10%).In 1266 (87%) out of the 1456 cases having completed the questionnaire, there was no protector in use. Typically, the situation was either unexpected or the protector had fallen off. In only 42 (3%) incidents had the conscript used a well-fitting hearing protector. In the remaining 142 (10%) cases there was a poor fit of the protector or another reason. In six questionnaires, no answer was given to this question.Tinnitus was the most prominent symptom, and the most typical hearing loss after AAT was seen at the frequency of 6 kHz. Tinnitus and other hearing symptoms were asked about separately from the conscripts in Group II. Tinnitus occurred after AAT in 201 (66%) out of the 306 subjects in this group, and other ear symptoms  in 144 (47%). Both types of symptoms occurred in 125 (41%) among the 306 conscripts.Noise caused damages for various group types of conscripts around the shot in 235 incidents. Most typically, two subjects (101 incidents), or 3–5 subjects (21–33 incidents depending on the group size) were exposed. Incidents with 6–12 subjects were encountered least frequently. For machine gun noise exposure, the most usual incident affected 2–6 conscripts (eight incidents). One example of an incident with a group of 27 conscripts without hearing protection was caused by six combat vehicles driving near the group and starting to shoot with machine guns with blank cartridges.We conducted a retrospective population-based 10-year study among all conscripts in the Finnish Defence Forces (FDF) during the years 1997–2003 and 2008–2010 to find out the frequency of acute acoustic trauma (AAT) after exposure to rifle-caliber noise exposure.The main finding was that most AAT-related hearing losses occurred after unexpected firing of an assault rifle. Most incidents occurred at the distance of less than two meters, with a neighboring conscript shooting and because no hearing protectors were used. The distances were evaluated by the victims who claimed to have adverse effects. The distances were sometimes later certified by training officers. The AATs were usually not serious (10–20 dB), although maximum hearing losses varied between 50 to 80 dB.The most prominent hearing loss after AAT in the present series typically affected the frequency band of 6 kHz. The same has been noted for the Finnish professional soldiers . The exaAlthough the hearing protectors are ordered to be used, the unexpected shots took place in circumstances where nobody in the training had hearing protectors. This forms a major challenge for the military training noise control. More importantly, it remains the responsibility of the training personnel to emphasize this even more in the rifle-handling of the conscripts. Unfortunately, false arguments still exist claiming that blank cartridges would not cause hearing loss. Developmental work to obtain blank cartridges with less noise emission from the muzzle of the rifle should be supported. There has been continuous product development in the muzzle brakes and wooden bullet breakers to enhance these efforts ,17.Communication hearing protectors are important in drill or shooting range activities, because there is always a risk of a shooting accident. There are many types of communication protectors when using earmuffs or plugs, and the variety is increasing.Although technical orders for training sessions have been established according to the safety precautions, noise-related accidents still occur, and these can cause even permanent hearing deficits. It seems that the human control factors should be emphasized even in crisis simulation conditions. The impact of a hearing disorder on a conscript’s future career and communication skills is evident and should thus not be underestimated.The present results are in accordance with earlier studies in Finland, Sweden and elsewhere ,18,19,20Around 0.7–1.5% of the conscripts experience an AAT during their service in the FDF. Vanmaele et al.  studied Our study includes certain limitations. Audiometric analysis results can occasionally be dependent on the attitude of conscripts at the end of their military service period as some individuals may seek compensation for their AAT-induced hearing loss. On the other hand, it is also possible that some conscripts did not complain of their hearing symptoms after noise exposure, and thus the damage was not registered. The amount of missing data was a minor factor in the present study. Furthermore, the possibility of combined effects on the background of the registered AAT, i.e., simultaneous heavy weapon shooting, blows on the ear, leisure time noise exposure, etc., remains. Our results on AAT-induced hearing loss and tinnitus are impossible to verify using a control group. The present series comprised 1674 of a cohort of 220,000 conscripts. In Finland currently about 70–80% of the male population undergo military training, and therefore it is difficult to obtain a control group for comparison. Furthermore, those with an earlier permanent sensorineural hearing loss or other major health problems get an exemption from military service.We conclude that around 0.7–1.5% of conscripts experience an AAT during their service with the Finnish Defence Forces. Most incidents occur after an accidental shot with an assault rifle, when persons around the weapon do not use hearing protection. Firing a blank cartridge as an accidental shot seems to be most typical at these occasions. Faults in weapon handling and carelessness regarding hearing protection in the battlefield military training form the basis for these. Training, control measures, and raising the awareness of possible consequences of ATT are the most important possibilities to reduce the incidence of these accidents.


Profound hearing loss is a disability that affects personality and when it involves teenagers before language acquisition, these bio-psychosocial conflicts can be exacerbated, requiring careful evaluation and choice of them for cochlear implant.To evaluate speech perception by adolescents with profound hearing loss, users of cochlear Implants.Prospective.Twenty-five individuals with severe or profound pre-lingual hearing loss who underwent cochlear implantation during adolescence, between 10 to 17 years and 11 months, who went through speech perception tests before the implant and 2 years after device activation. For comparison and analysis we used the results from tests of four choice, recognition of vowels and recognition of sentences in a closed setting and the open environment.The average percentage of correct answers in the four choice test before the implant was 46.9% and after 24 months of device use, this value went up to 86.1% in the vowels recognition test, the average difference was 45.13% to 83.13% and the sentences recognition test together in closed and open settings was 19.3% to 60.6% and 1.08% to 20.47% respectively.All patients, although with mixed results, achieved statistical improvement in all speech tests that were employed. Cochlear implants are the treatment of choice for patients with severe to profound sensorineural hearing loss which does not respond to conventional hearing aidsWhen treating teenagers with prelingual hearing loss, the long time of hearing deprivation reduces considerably the opportunity of gaining speech recognition after surgery. Therefore, when indicating cochlear implant to a heterogeneous group in terms of etiology, hearing impairment duration, cognition and language, we must take into account the parents’ expectations, family dynamics, the patient's wishes, his relationship with his social group, what are the implications in his identity - such as the patient's degree of maturity and affective-emotional conditionsOur opinion has changed with the progress achieved in speech processors, which are now able to provide some degree of speech recognition in this population. Nonetheless, it is necessary to review our concepts of benefit and good results, thus restructuring the pre-implant evaluation which started to emphasize language assessment, social insertion and expectations concerning the cochlear implant, besides enjoying hearing with a hearing aidThe few studies about the benefits brought about by the cochlear implant to teenage patients have varied resultsThis study was carried out in the Cochlear Implant Ward of the Otology Group of the University of São Paulo Medical School, and it was approved by the Ethics in Research Committee of the institution, under protocol # 1061/08.-Age between 10 and 17 years and 11 months-Prelingual hearing loss-Severe to profound sensorineural hearing loss-All the patients used hearing AIDS before the cochlear implant.25 adolescents were included with the following criteria:The assessment was carried out individually with each patient, before the cochlear implant and 2 years after they started using it, and during the two year os CI use, the patients were followed by an audiologist for the practice and development of hearing skills.Only 2 of the 25 patients could not be assessed after 2 years of using the CI, because they did not come to the appointment. Thus, in the result analysis we included 23 adolescents.Fifteen of the participants were females and 8 were males .Table 1DRoutinely, as part of patient selection, in order assess performance and to obtain the data necessary for programming, all implanted patients were submitted to speech perception tests before being submitted to cochlear implant and six months after the activation of the device. This test is a prospective quantitative analysis in which the selected patients were submitted to speech perception tests before and 6, 12 and 24 months after the cochlear implant device activation. For this study, we considered the results obtained in the tests after 24 months of use in order to compare it with the pre-implant results.four choice, vowel recognition, closed set sentences recognition, open set sentence recognition, in the audio-visual and auditory modes. All the tests had a maximum score of 100% and minimum of 0%, and each correct answer or wrong answer corresponds to a percentage which varies according to the total number of sentences or words existing in the test; for instance, the closed set sentences recognition test is made up of 10 sentences, thus, each one corresponds to a total of 10%.The speech perception test was carried out live and the complete evaluation protocol was described by Gómez et al.Statistical Package for Social Sciences (SPSS) software, version 16.0 for Windows . We analyzed the correlation between the speech recognition tests through the use of the Pearson's and Spearman's correlation coefficients. The pre and post cochlear implant scores comparison from the speech recognition tests were carried out using the non-parametric Wilcoxon test for paired samples. To do that, we considered the statistically significant differences, having p values below 0.05.The statistical analysis was carried out by the four choice test before and after the cochlear implant was of 46.9% and after 24 months of device use, this mean value went up to 86.1%. In the vowel recognition test, the mean values were between 45.13% to 83.13%. in the closed set sentences recognition test, the mean value before the cochlear implant was 19.3% and after 2 years, the mean was 60.6%. In the open set sentences recognition test, the mean percentage of correct answers before the implant was 1.08% and after 2 years, this value went up to 20.47%. four choiAdolescence is a very particular stage of development. The psychological changes which happen at this time, together with the body changes, cause numerous bio-psycho-social conflicts in the individual; thus, it is necessary to be cautious in the assessment and selection of candidates to cochlear implant, especially in individuals with prelingual hearing loss, in whom hearing results are very heterogeneous.The main goal of cochlear implants in adolescents with prelingual hearing loss is to audiologically enable them to perceive and recognize speech, and the hearing thresholds in all the individuals after the cochlear implant were enough to have access to it.There is a consensus in assessing the results from the speech perception tests 12 months after continuous use of the cochlear implant, when there would already be a learning curve flattening We could also observe this, because despite the individuals being adolescents with prelingual hearing loss, the results vary among themselves - which can be justified by another study between the differences in speech recognition performance differences considering characteristics such as: time to diagnosis, etiology, onset of hearing aid use, speech therapy strategy and emotional characteristicsRegardless of the results obtained in the four choice, vowel recognition and closed and open set sentence recognition, all individuals reported increases in selfconfidence and improvement in the general well-being after they started using the cochlear implant. This fact is associated with the idea of improving the quality of life of these individuals, and being teenagers, this represents a very important issue in the psycho-social aspect of their lives. Psychological status is also a determining factor for satisfactory results or not in the speech perception tests of adolescents•All the patients obtained sufficient hearing thresholds in order to have Access to speech sounds;•All the patients, although with heterogeneous results, obtained statistical improvements in all the speech tests employed.Based on the analysis of the results from the tests made with the 23 adolescents, we have concluded that:


PIK3CA gene. It is unilateral and involves hypertrophy of both the soft and hard tissue structures on the affected side of the face. This commonly results in early eruption of the teeth, hypertrophy of the facial bones, macroglossia, and proliferation of the parotid gland. Less than 80 cases of CILF have been reported in the literature so far. Treatment modalities include liposuction and surgical excision. However, since the hallmark of CILF is mutation in the PIK3CA gene, PI3K inhibitors may play a therapeutic role in CILF. We report a case of an 8-year-old boy with recurrent CILF of the scalp and nose, with PIK3CA H1047R mutation. We discuss the differential diagnoses, clinical outcomes, and management of this rare entity.Congenital infiltrating lipomatosis of the face (CILF) is a rare, congenital, nonhereditary facial overgrowth due to post-zygomatic activating mutations in  PIK3CA gene, constituting a part of PIK3CA-related overgrowth syndrome. This causes mature lipocytes to invade into the adjacent tissues in the facial region [FGFR3 gene has also been shown to instigate a role in the etiopathogenesis of CILF [Congenital infiltrating lipomatosis of the face (CILF) is a rare, congenital, nonhereditary, unilateral facial overgrowth . Althougl region ,3. The F of CILF . This lePIK3CA H1047R mutation.CILF was first described by Slavin et al. in 1983 . HistoloAn 8-year-old boy was brought into our institution with facial asymmetry progressively evolving since birth. He had a similar history of a subcutaneous scalp mass at birth and another mass at the right upper eyelid, glabella and scalp at the age of 2, which was removed surgically without any complication. Now, the patient presented with a recurrent mass involving the scalp and nose. There was no significant family history or any siblings with similar presentation. Physical examination showed an ill-defined soft tissue mass in the forehead extending to the scalp and inferiorly to the nose bridge and right lateral aspect of the nose . The patHead and face computed tomography scan and magnetic resonance imaging were performed and showed an enlarging soft tissue lesion measuring 9.7 × 7.4 × 3.1 cm in the forehead towards the right side extending to the parietal scalp. There was no involvement of the underlying skull bone, brain, or ventricle. The lesion was characterized primarily by fat signal with areas of soft tissue signal in the periphery, traversing the fatty component . Spiral Surgical resection of the mass via a bicoronal incision on the scalp was performed. Intraoperatively, dissection was carried down to the periosteum underneath the area of the level of the galea and the mass was noted. Careful and slow dissection was done to ensure complete removal of the mass and to preserve the intactness of the overlying skin and subcutaneous tissue. Due to the infiltrative nature of the mass which showed extensive local infiltration into the muscles, skin and galea, it was removed via a piecemeal approach. The patient tolerated the procedure well and was sent to recovery in stable condition.PIK3CA H1047R gene , supporting the diagnosis of congenital infiltrative lipomatosis of the face. The tumor was microsatellite stable and there was no increase in tumor mutational burden (TMB) (0 mutation/Mb).Macroscopically, multiple irregular fragments of focally hemorrhagic yellow to brown soft tissue with yellow-pink lobulated cut surface and focal white-tan fibrous elements. Histopathological examination showed that the mass consisted predominantly of mature adipose tissue with thin and thick, irregular fibrous septae . No atypPIK3CA gene in CILF, it is now considered part of PIK3CA-related overgrowth syndrome [PIK3CA gene mutation is rare. A review of the literature of select reported cases thus far has identified frequent PIK3CA hotspot mutations including H1047R, which is the most frequent drive mutation hotspot, as in our case, and other hotspots such as H1047L, E453K, E452K, and E542K  is a rare, congenital, nonhereditary facial overgrowth first described in 1983 by Slavin et al. . There asyndrome . Neverthnd E542K .PIK3CA mutations,” “Congenital Infiltrating Facial Lipomatosis,” and “Facial Infiltrating Lipomatosis.” The subsequent literature review showed that prior to 2013, no reported cases of CILF with a confirmed PIK3CA mutation are found. This is likely due to the more recent discovery of PIK3CA as a driver mutation for pathogenesis of CILF, and the natural delay in translating this discovery towards actual clinical testing performed to confirm the diagnosis in studied patients. Thus, previously published cases not including confirmed PIK3CA mutations were excluded from this review. The review of literature produced 16 case reports in a total of 7 publications. While no language restrictions were used, the utilized articles were all published in English.Additionally, a literature review was performed using PubMed , EMBASE (Elsevier), and CIHNAL (EBSCO) using multiple variants of common search keywords (MeSH terms) such as “CILF,” “FIL,” “PIK3CA mutations. This collated search revealed the importance of increasing molecular testing in patients with suspected CILF, as the vast majority of recently published literature on CILF unfortunately does not include molecular testing as a confirmatory approach. While the knowledge of this mutation existing in our patients does not necessarily change the surgical approach and management of CILF, it does reveal that potential various molecular therapeutics may be used if they happen to interrupt the aberrant PIK3CA pathway involved in the pathogenesis of CILF. These new potential therapeutics will be briefly described later in our discussion. We hope and anticipate that further research utilizes molecular screening tools such as comprehensive genomic profiling or techniques such as the ones listed on PIK3CA mutations without explicitly reporting the molecular testing methodologies performed. However, of the publications that reported PIK3CA mutations as the cause of the CILF, 6 cases performed PCR testing to identify the mutations, and the other 6 cases performed ddPCR testing. In terms of the types of mutation, all except one case reported specific mutation detected. Missense substitution p.H1047R appears to be the most frequently found at 7 out of 15 cases (7/15)), followed by the second most frequent mutation reported being the missense substitution mutation of p.E542K, which was found in 4 of the 15 cases (4/15). The least frequent mutation reported in this review was p.H1047L, which was found in 3 out of 15 cases (3/15).Previous research has notably shown that CILF does not show any sex or side predilection. In our findings shown on Clinically, CILF presents as unilateral, progressive, painless enlargement of the soft and bony facial tissue structures and occurs at birth or in early childhood (under the age of one year). Notably, CILF does not show any sex or side predilection. Due to its extensive and infiltrative growth pattern, it may be associated with orodental issues such as macrodontism, early eruption of deciduous and permanent teeth, and facial deformities, which entail a significant social and psychological impact . HistoloPIK3CA gene are identified in CILF as well as other benign overgrowth syndromes, which are collectively termed PIK3CA-related overgrowth syndrome [PIK3CA is a gene that encodes p110α, a catalytic PI3K subunit that has a physiological role in growth and development and frequently displays pathological hyperactivation in cancers [PIK3CA with asymmetric overgrowth, exemplifying a mosaic genetic activation of p110α. Furthermore, PIK3CA hotspot mutations were also identified in benign skin lesions such as epidermal nevi and seborrheic keratosis [Postzygomatic activating mutations in the syndrome . PIK3CA  cancers . With th cancers ,16,17. Heratosis , represeeratosis . This steratosis .PLAG1 rearrangements or copy number gain, which is absent in our case. Lastly, the absence of lipoblast, atypical adipocytes, and mitosis can help to rule out a diagnosis of liposarcoma, which more commonly affects older population, and shows a predilection to involve the trunk and extremities.The diagnosis of CILF is mainly clinical. Imaging studies such as ultrasound, computed tomography scan, and magnetic resonance imaging may help to detect the presence of abnormal vascularity or any involvement of underlying bone and soft tissue structures. Histopathological examination of the biopsied or excised tissue helps to rule out other possible differential diagnoses in the facial region such as lipoma, lipoblastoma or liposarcoma. Lipoma is a common benign neoplasm of adipocytic differentiation and usually presents in adulthood. Lipoma is usually well encapsulated and follows a relatively indolent course, whereas CILF is non-encapsulated with ill-defined borders and tends to infiltrate extensively into surrounding tissues. Lipoblastoma, another benign entity which is commonly seen in young children, shows similar histopathology and is characterized by sheets of mature adipocytes, separated by delicate fibrovascular septae. However, lipoblastoma typically involves the trunk and extremities and rarely arises in the head and neck region. Furthermore, the diagnosis of lipoblastoma can be supported with PIK3CA mutations, associations with malignancies are rare. The only reported malignancy in PIK3CA-related overgrowth syndrome is Wilms tumor (nephroblastoma) which is identified in four out of 200 patients with PIK3CA-related overgrowth syndrome [PIK3CA driver mutations can confer a selective growth advantage during cancer development, the mutation alone is not sufficient for cancer initiation or maintenance [PIK3CA-related overgrowth syndrome and, likely, CILF.CILF is an indolent benign tissue overgrowth with a high risk of recurrence, requiring multiple debulking surgeries. Of note, although CILF is associated with ntenance  and requntenance ,23, an iIn a review of literature by Li et al., incorporating all cases of CILF reported until 2017, 59 patients  were found [PIK3CA-associated adult cancers among PIK3CA-related overgrowth syndrome patients, cooperating mutations might still contribute to cancer development in this population of patients [The diagnosis of this pathological entity is based on the clinical history, clinicopathological manifestations, imaging features from computed tomography of the head region, and genetic profiling of the excised tissue. Although there are not sufficient data about an increased risk of patients .PIK3CA gene mutation in CILF renders inhibitors of PI3K such as Alpelisib, which are currently under study in clinical trials for many cancers including lymphoma, a potential therapeutic option for CILF patients [Treatment modalities currently available for CILF include mainly liposuction and surgical excision . Althougpatients .PIK3CA gene mutation. This case highlights the need for heightened awareness of this rare disease among pathologists as well as the need to perform molecular studies on “seemingly benign”-appearing mature adipose tissues from the face and scalp of a child. In conclusion, we report a case of recurrent CILF in an 8-year-old boy with