The otoscopic view is of the right ear of a 27-year-old storekeeper whose chief complaint was hearing loss (figure). The appearance of his contralateral ear was similar. A severely retracted atrophic drum was adherent to the medial wall of the tympanic cavity. The handle of the malleus was retracted and was probably in contact with the promontory. In the anterior portion under the drum, the protympanum appeared to be well ventilated. The retraction was molded against the walls of the hypotympanum, forming a widely open pocket in which some products of desquamation had accumulated. Pure-tone audiometry indicated a 40-dB air-bone gap. Politzerization was not possible, indicating that the tympanic membrane was fixed.
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What should the otologist recommend for this patient? It is tempting to propose a surgical solution. Successful and long-lasting results have been achieved with extremely reliable techniques for tympanic membrane repair, including (1) the use of fascia, perichondrium, or cartilage to prevent subsequent retraction, (2) ossicular transposition, and (3) biocompatible prostheses. However, the long-term results of any such procedures in this type of case are unpredictable. We do not know whether the inflammatory phenomena associated with the underlying disease and the problems of ventilation of the middle ear have been controlled. There is a risk that any improvement will be only temporary and that the basic pathophysiology will recur. Because there is no means of observation and no test available that can predict outcome in these cases, the otologist must consider surgical options with the utmost prudence. There is no imminent risk of complication in selecting a nonsurgical approach. Placing a hearing aid is a valid noninvasive alternative because it ensures an immediate positive result for the patient.
Moreover, because the patient's condition was bilateral, another approach would have been to attempt reconstruction of one ear only. Such a decision must rest with the patient, who must be made thoroughly aware of the uncertainty of the outcome.
Christian Deguine, MD
Jack L. Pulec, MD ([dagger])
From Gap, France (Dr. Deguine), and the Pulec Ear Clinic, Los Angeles (Dr. Pulec).
([dagger]) The late Dr. Pulec was editor in-chief of EAR, NOSE & THROAT JOURNAL from 1992 through 2003.
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Aagenaes syndrome