Mondo Description Any X-linked nonsyndromic deafness in which the cause of the disease is a mutation in the SMPX gene.

Uniprot Description A non-syndromic form of sensorineural, progressive hearing loss with postlingual onset. In affected males, the auditory impairment affects initially high-frequency hearing. It later evolves to become severe to profound and affects all frequencies. Carrier females manifest moderate hearing impairment in the high frequencies.