Usher Syndrome Type Iic Gpr98 Pdzd7 Digenic C3148929
A rare digenic form of Usher syndrome involving mutations in the GPR98 and PDZD7 genes. Causes retinitis pigmentosa and hearing impairment. Management involves hearing and vision support tailored to individual symptoms.
Key facts at a glance
- ICD-10
- Q89.9
- ICD-11
- LA00.0
- Body system
- congenital
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(congenital)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Profound sensorineural hearing loss
(ear)Retinitis pigmentosa (night blindness)
(eye)Vestibular dysfunction (balance problems)
(ear)Severe visual impairment (legal blindness)
(eye)Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy