Ocular, auditory and behavioral management are the most common areas of intervention and treatment for patients with<unk> . For ocular (eye) management, often patients already suffer from complete retinal detachment at birth, or by the time of diagnosis, so surgical intervention is often not offered. However, there is some evidence for the benefit of early surgery or laser therapy for cases where retinal detachment is incomplete. Surgery may also be used to treat increased intraocular pressure and in rare cases enucleation (removal) of the eye is considered to control pain. A high proportion (85–90%) of individuals with<unk>  experience progressive hearing loss in their second decade of life. In most cases, use of hearing aids has been shown to be effective into middle or late adulthood. For more significantly impaired hearing, cochlear implants may also be considered. 30-50% of individuals with<unk>  have been reported to present with developmental delay or cognitive impairment. Additionally, behavioral issues have also been reported. Supportive intervention and therapy, for example working with speech and language therapists and occupational therapists, can be used to maximize educational opportunities for these individuals. Furthermore, training of teachers and school counselors on how to best support children with vision and hearing impairment can be extremely beneficial. Routine monitoring of individuals with<unk>  is recommended to best manage the disease. This includes regular follow-up with an ophthalmologist, even when vision is severely compromised. Additionally, due to the high proportion of individuals with<unk>  who develop hearing loss, regular monitoring of hearing loss is beneficial to allow any hearing loss to be detected early and then correctly managed. More recently, the use of dual sensory clinics has been proposed to provide improved care to patients living with conditions such as<unk> . For example, Great Ormond Street Hospital (GOSH), London are building a new Sight and Sound center, with the aim of improving the patient experience for individuals with conditions such as<unk> . The benefits of dual sensory clinics include improved communication between the different health care professionals (HCPs) involved in management of<unk>  (e.g. ophthalmologists and audiologists) as well as allowing more consistent training of staff on best practices for managing and interacting with individuals suffering from sensory impairment. Individuals with<unk>  can often feel isolated from society due to difficulties in communication. In cases where hearing loss is also experienced, this psychological burden has been shown to increase. For example, a number of<unk>  patients have been reported to experience transient depression correlating with the onset of hearing loss. Because of this, the provision of emotional support to individuals with<unk>  can be as important as clinical treatment strategies in terms of improving their quality of life and reducing disease burden.
Norrie disease
Waldmann disease
Karak syndrome
Camisa disease
Batten disease
Leber congenital amaurosis
Gleich's syndrome
Zaki syndrome