Konigsmark Syndrome

Inherited progressive sensorineural hearing loss and the presence of malformed middle ears. Other features include mental retardation, narrow palate, and hypogonadism.

Autosomal Dominant, Nonsyndromic Sensorineural Deafness; Hereditary Low-Frequency Hearing Loss; Konigsmark-Hollander-Berlin Syndrome; Konigsmark-Knox-Hussels Syndrome.

Autosomal dominant. Gene map locus is 5q31.

Short increment sensitivity index (SISI) examination suggests a cochlear lesion as the source of the hearing loss.

Familial occurrence of low-frequency sensorineural hearing loss.

Sensorineural hearing loss that shows moderate variation in affected persons. In general, the hearing loss begins in childhood, but postlingual (after language and speaking have been acquired), affecting first the low frequencies and progressing slowly to involve all frequencies in the latter decades of life.

No specific precautions associated with this syndrome other than the usual preoperative preparation for an anesthesia.

Anesthetic considerations for any individual affected with this syndrome are those associated with the surgical procedures rather than the syndrome itself.

No known specific pharmacological implications.