Cystic cochleovestibular anomaly presenting with congenital deafness and recurrent bacterial meningitis in childhood
A 5-year-old girl born out of a nonconsanguineous parentage, deaf and dumb from birth with other milestones being normal, was brought to our hospital for evaluation of three episodes of bacterial meningitis.\nDuring the first episode, the child presented to a pediatric general hospital (GH) with high-grade continuous fever and vomiting for 3 days followed by altered sensorium for 2 days. On examination, the child had mild left orbital cellulitis, was drowsy, and meningeal signs were present without features of raised intracranial pressure or focal deficits. Routine investigations were done which showed mild anemia; computerized tomography (CT) of brain showed diffuse cerebral edema with left ethmodial sinusitis and orbital cellulitis on the left side. Lumbar puncture (LP) revealed turbid cerebrospinal fluid (CSF) with 350 cells (90% polymorphs, 10% lymphocytes), protein of 780 mg/dl and glucose less than 10 mg/dl. Gram stain was negative, but CSF culture showed growth of Streptococcus pneumoniae sensitive to penicillin, gentamycin, cefotaxime, ceftriaxone, vancomycin, and ciprofloxacin. She was treated with sensitive antibiotics for 2 weeks and she recovered. She was referred to our institute by the pediatrician who treated her during the first episode, but the patient’s father got her discharged within 12 hours because of social problems.\nShe presented once again to the same pediatric hospital after 4 months with high-grade, continuous fever and headache of 3 days duration. On examination, she was conscious and had meningeal signs with no focal deficits. Routine investigations were normal except for mild anemia. CSF showed 250 cells (100% polymorphs), protein of 65 mg/dl, and glucose less than 20 mg/dl. Gram stain was negative; culture did not grow any organism. She received injectible crystalline penicillin and ceftriaxone for 14 days and became fully asymptomatic.\nApproximately 1 month later, she once again developed high-grade, continuous fever, vomiting, and altered sensorium of 2 days duration. This time, the child was referred to our hospital for evaluation and treatment, without being admitted at GH. On examination, the child was drowsy and meningeal signs were present without any features of raised intracranial tension or focal deficits. Lumbar puncture revealed turbid CSF with cell count of 2000 (90% polymorphs), protein of 168 mg/dl, and glucose of 23 mg/dl. Pneumococcal antigen was positive in the CSF. Blood and CSF cultures were negative. She was treated with ceftriaxone for 2 weeks in meningitic doses and improved totally. There was no history of any ear discharge, rhinorrhea, head injury, and recurrent respiratory or skin infection. On examination, there was no splenomegaly, sinus, or tuft of hair over spine. Her routine investigation showed mild anemia, leukocytosis, normal biochemistry, and nonreactive HIV test. Brainstem-evoked auditory responses were absent bilaterally with audiometry suggestive of bilateral severe sensory neural hearing loss.\nShe was evaluated with high-resolution computerized tomography (HRCT) scan of the temporal bone and magnetic resonance imaging (MRI) of the brain. On the left side, the cochlea was showing a featureless single cavity. There was complete absence of modiolus and interscalar septa. The vestibule was grossly dilated. The cystic cochlea and vestibule together gave a “figure-of-eight” appearance. The lateral semicircular canal was dysplastic and dilated. Superior and posterior semicircular canals were normal. Internal auditory canal (IAC) was dilated and contained normal facial and vestibulocochlear nerves. The vestibular aqueduct was normal. The left tympanum and the mastoid air cells were filled with soft tissue density, indicating either CSF leakage or otitis media with mastoiditis [Figures and ].\nThe cribriform bony plate between the cochlea and IAC was deficient, producing an open fundus communicating with the cochlea. This could be a probable site of communication between the subarachnoid space and inner ear. CT cisternography, which reveals the abnormal transotic CSF leak, was not performed in our case. However, the oval and round windows were wider. These could be the probable sites of communication between the inner ear and middle ear. The middle ear ossicles (malleus, incus, and stapes) and tympanic membrane were normal. The right inner ear also revealed similar abnormality. However, the tympanum and mastoid were normal.\nWith these features, the patient was diagnosed as a case of congenital deaf-mutism with RBM, cystic vestibulocochlear anomaly, and probable infection arising from left middle ear cavity. She was evaluated by the ENT surgeon, and after 1 month of the last episode, she underwent left middle ear exploration and a communication at the stapes foot plate with inner ear was identified and closed. The patient has had no recurrences from the last 1½ years.