Ossicular Abnormalities in Down's Syndrome

Abstract

Down's syndrome has been associated with hearing loss and otitis media. Because of difficulty in examination, however, there have been few detailed otologic studies on this population. An understanding of the nature and frequency of ear disease in Down's syndrome is important, since it is common and occurs in more than one in every 600 live births. To define the aural manifestations of Down's syndrome, complete otologic and audiometric examination was performed on 107 consecutive patients. This included micropneumatic otoscopy, pure-tone and speech audiometry, impedance tympanometry, and, in some cases, electronystagmography. Deficient hearing was found in 64% of these patients, and of these hearing losses, 83% were conductive. Surprisingly, middle ear effusion or tympanic membrane perforation accounted for only 60% of the conductive hearing losses. This finding prompted us to examine five temporal bones of children with Down's syndrome. These histologic sections revealed middle ear abnormalities including fixation and superstructure deformity of the stapes and dehiscence of the fallopian canal. Operative findings in 16 procedures on patients with Down's syndrome and conductive hearing loss support those findings.

Down syndrome: Common otolaryngologic manifestations

“…While Balkany et al [1979b] agreed with the need for aggressive medical and surgical management of middle ear infections, they found little improvement in conductive hearing loss in patients with DS who underwent reconstructive surgery for ossicular abnormalities. They suggested a more conservative course such as amplification in patients who continued to have a conductive hearing loss following treatment of their chronic ear disease [Balkany et al, 1979b].…”

“…Answers to these questions are not yet published. Balkany et al [1979b] reported that 83% of the hearing loss seen in patients with DS was due to a 'conductive' or middle ear abnormality. Sixty percent of this conductive hearing loss was attributed to chronic ear disease such as middle ear infections or tympanic membrane perforations, and the other 0% was felt to be due to ossicular abnormalities with malformations found on temporal bone studies.…”

Mastoid abnormalities in down syndrome

“…Of the group of 22 children with Down syndrome, 8 children had normally ation during examination [1]. Abnormalities of the pinna are very common in Down syndrome [1].…”

“…Of the group of 22 children with Down syndrome, 8 children had normally ation during examination [1]. Abnormalities of the pinna are very common in Down syndrome [1]. It has been suggested that the hypotonia associated with Down syndrome may also affect the tensor veli palatini and cause Eustachian tube dysfunction [2].…”

“…It has been suggested that the hypotonia associated with Down syndrome may also affect the tensor veli palatini and cause Eustachian tube dysfunction [2]. This, combined with the common occurrence of a narrow external auditory meatus, might result in a high incidence of undetected middle ear effusion [1,2]. In the series of Balkany and Mishke, deficient hearing was found in 64% of their patients and in 83% of these, the loss was conductive and mostly due to middle ear effusion [1].…”