Ossiculoplasty in congenital hearing loss.

We have presented an extensive review of congenital ossicular anomalies. These deformities occur in combination with one another or as isolated problems with equal frequency. They are so rare that even the busiest otologists have limited clinical experience with them. The astute otologist will keep these facts in mind when assessing congenital middle ear problems in the office and in the operating room. Careful, detailed history taking may reveal the presence of familial conductive hearing loss or other congenital syndromes. Complete head and neck examination as well as observation of the extremities may provide clues to the otologic diagnosis. Microscopic examination of the ear reveals abnormalities of the tympanic membrane, malleus, and incus. Audiometric and radiologic evaluation augments physical examination. At surgery, the otologist must be prepared to perform a variety of possible surgical procedures, from traditional ossiculoplasty and stapedectomy to fenestration. Hearing aids are an option for patients with congenital ossicular anomalies, and the possibility of inoperability must be kept in mind; the patient must be prepared for this eventuality. Congenital ossicular anomalies present a challenge to the otologic surgeon, and their successful treatment, the greatest reward.