Fred H. Linthicum

Sensorineural Hearing Loss Associated with Systemic Lupus Erythematosus

Because our recent clinical experiences suggested a possible association between hearing loss and systemic lupus erythematosus (SLE), we prospectively studied the hearing of 30 patients hospitalized because of exacerbation of SLE. Twenty-nine of the 30 patients were receiving immunosuppressive therapy at the time of testing. We found an 8% incidence of substantial, previously undetected hearing loss without attributable cause. Hearing loss could not be correlated to age, sex, disease activity, organ-system involvement, laboratory test abnormalities, or duration of symptoms of SLE. Otolaryngologists treating patients with unexplainable hearing loss—particularly if it is sudden, fluctuating, or rapidly progressive—are alerted to the possibility of underlying systemic autoimmunity. We advise physicians who frequently treat patients with SLE to include questions on hearing in the review of systems and to refer the patient with hearing complaints for thorough otologic evaluation.

Long-Term effects of silastic® sheeting in the middle ear

Silastic® sheeting is used to restore a mucosally lined middle ear space and to prevent mucosal adhesions between the medial surface of the tympanic membrane and the promontory after surgical procedures involving the removal of middle ear mucosa. To determine the long‐term (1.2 to 21.2 years) effects of Silastic sheeting in the middle ear, six temporal bones from patients with permanent Silastic sheeting in the middle ear cavity after single‐stage tympanoplasty were examined. In all six bones, no histologic evidence of foreign body reaction, rejection, or chronic inflammation in the middle ear surrounding the silicone sheeting was observed. Furthermore, the middle ear space was lined by normal‐appearing, continuous middle ear mucosa. In four temporal bones, there was no evidence of significant submucosal fibrosis. In two bones, significant submucosal fibrosis was attributable to concurrent middle ear disease rather than to the presence of the silicone. It is concluded that Silastic sheeting in the middle ear after single‐stage procedures may prevent adhesions between mucosal surfaces. Given eustachian tube function, Silastic promotes a mucosally lined, aerated middle ear cavity without apparent foreign body reaction, rejection, or chronic inflammatory response.

Computer-Aided Reconstruction of the Endolymphatic Sac

Computer-aided three-dimensional reconstruction techniques along with surface area and volume determinations have been applied to the intraosseous portion of Ihe human endolymphatic sac. Three-dimensional reconstruction has revealed that the lumen of the sac is a series of parallel tubules rather than folds. Surface-to-volume ratio determinations may reveal that the resorptive capacity of epithelium in this portion of the sac is significantly reduced in patients with Menieres disease.

Arterial Supply of the Human Endolymphatic Duct and Sac

The arterial anatomy of the endolymphatic duct and sac was studied in vascular casts of methyl methacrylate of six human heads. The chief source of arterial blood supply to the endolymphatic duct and sac appeared to be the occipital artery. Arterioles entered the bone of the mastoid process. Arterioles in bone, the walls of the sigmoid sinus, and the posterior fossa dura coursed medially to supply the endolymphatic sac. The orientation of arterioles tended to be along the long axis of the endolymphatic duct and sac, whereas venules were more likely to be circumferentially oriented. Arterioles arising from dural vessels divided into deeper branches, which supplied periductal connective tissue, and superficial branches, which entered canaliculi of the vestibular aqueduct. Gross anatomic findings were confirmed by histologic examination of temporal bones.)

Endolymphatic pseudohydrops of the cochlear apex

OBJECTIVE: To demonstrate that what appears to be endolymphatic hydrops of the apical scala media is normal anatomy. STUDY DESIGN: Computer-generated three-dimensional reconstruction of the cochlear apex and tabulation of the number of cases with arched Reissners membranes (pseudohydrops) versus flat membranes. SETTING: Temporal bone laboratory consisting of 809 documented pairs of temporal bones. SUBJECTS AND METHODS: Archival temporal bone sections from 107 bones (65 patients) were used to determine the percentage of arched (pseudohydrops) versus flat Reissners membranes. Two bones, one of each membrane shape, were randomly selected for computer-generated three-dimensional reconstructions showing the cochlear apical anatomy. RESULTS: An arched Reissners membrane was found in 48.6 percent of bones. In the cochlear apex, Reissners membrane appears to be distended, simulating hydrops, due to its transition from a conical structure to a triangle bounded by the basilar membrane with the organ of Corti, the stria vascularis, and Reissners membrane. Membrane findings were similar in both ears in 73.8 percent of the bilateral cases studied. There were no significant relationships between membrane type and clinical characteristics. CONCLUSION: What appears to be endolymphatic hydrops of the cochlear apex is the transition area of the cochlear duct from a conical shape at the extreme apex to the triangular shape found in the rest of the cochlea. The appearance of distension is dependent upon the cochlear length and the level of the microscopic section.

Pathologic correlates of electronystagmographic tracings

Electronystagmographic tracings were correlated with the histopathologic findings in the peripheral vestibular system of 66 temporal bones of 34 patients who complained of dizziness. Although some findings (e.g., spontaneous nystagmus, unilaterally reduced caloric response, and directional preponderance) seemed to have doubtful value in localizing vestibular system disease, other findings indicated a peripheral site of the vestibular lesions. Bilaterally reduced or bilaterally absent caloric responses were consistently associated with pathologic changes in the peripheral vestibular system. Similarly, a unilaterally absent caloric response consistently indicated peripheral disease. On the other hand, positional nystagmus and bilaterally equal caloric responses seemed to indicate the absence of a peripheral site of disease in the vestibular system. Although there is no reliable way to differentiate end organ from vestibular nerve lesions, the latter were more commonly associated with positional nystagmus and absence of a caloric response. The direction of nystagmus beating correlated with the side of the diseased peripheral site in two of four patients with spontaneous nystagmus, in five of seven with positional nystagmus, and in all with unilateral reductions in or absence of caloric responses. Electronystagmographic findings in the present study seemed to reflect diffuse rather than localized pathologic changes in the peripheral vestibular system.

Intracochlear glandular schwannoma

Benign glandular schwannomas are rare, with 8 cases having been reported in the literature. The tumors have been found on the extremities, trunk, and scalp, but none have been reported as being associated with the ear, a common location for sporadic schwannomas. Intralabyrinthine and cochlear schwannomas, such as illustrated in this case, were rarely described until the advent of magnetic resonance imaging, when reports of the tumor became quite common. However, no cases of benign glandular schwannomas in the ear have been described previously.

Hypoplastic endolymphatic sac, hydrops, and Mondini deformity: a case report.

The Mondini deformity of the inner ear is usually associated with a large vestibular aqueduct and endolymphatic sac. The authors present a case with a hypoplastic sac and endolymphatic hydrops, which are presumed to be the cause of the Menieres syndrome symptoms that occurred in mid‐life.