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Shan-Rong Shi, MD

Arch Otolaryngol. 1985;111(2):119-121.

Abstract
• The principal features of the otopalatodigital syndrome are hearing loss, cleft palate, and skeletal dysplasia of the hands and feet. The right temporal bone was acquired from a boy with this syndrome who died at the age of 2 years. Behavioral audiometry had indicated a conductive hearing loss, with probable near-normal sensorineural function; brainstem evoked response audiometry indicated a mild sensorineural hearing loss. Histologic studies of the temporal bone revealed dysmorphic features in both the middle ear and the bony labyrinth. The ossicles were deformed, the stapes was fixed, and no round window was present. A defect of the modiolus resulted in a wide communication between the subarachnoid space of the internal auditory canal and the scala vestibuli. These anomalies would clearly have frustrated any attempt to improve the patient's hearing through reconstructive middle ear surgery.

(Arch Otolaryngol 1985;111:119-121)

Author Affiliations
From the Department of Otolaryngology at Harvard Medical School and the Massachusetts Eye and Ear Infirmary, Boston. Dr Shi is now with the Department of Otolaryngology, Sichuan Medical College, Sichuan, People's Republic of China.

Footnotes
Accepted for publication Aug 3, 1984

Reprint requests to the Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, 243 Charles St, Boston, MA 02114 (Harold F. Schuknecht, MD).

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