Human autoantibodies and monoclonal antibody KHRI-3 bind to a phylogenetically conserved inner-ear-supporting cell antigen.

Autoimmunity is thought to be one cause of sensorineural hearing loss (SNHL). Sera from patients with rapidly progressive hearing loss have been shown to contain antibodies to a 68-kD protein in heterologous inner-ear tissue. Using guinea pig inner-ear tissue as the antigenic substrate and either Western blot or immunofluorescence (IF) or both, we tested sera from 74 patients suspected to have autoimmune hearing loss for inner-ear antibodies. Sera from 73 patients were tested by Western blot, and sera from 36 were tested by IF. Thirty-seven of 73 (51%) had antibody to a 68-70-kD protein by Western blot. Sera positive by IF stained supporting cells with a staining pattern like that previously observed with the KHRI-3 monoclonal antibody. There was concordance between Western blot and IF assays. Of 36 patients tested by both assays, 29/31 (94%) that were positive in Western blot were also positive by IF, three were negative by both tests, and two each were positive by one assay but negative by the other. Absorption of patient sera with human inner-ear tissue removed antibody reactivity to the guinea pig supporting cells, indicating that the antigen detected by the autoantibody is also present in the human inner ear. Absorption with an equal volume of white or red blood cells from the tissue donor did not remove the antibody reactivity to inner ear, showing that the absorption by inner-ear tissue is specific. Sera from three patients positive in both assays also stained a 68-70-kD inner-ear protein immunoprecipitated by the KHRI-3 monoclonal antibody, indicating that the monoclonal and human antibodies recognize the same antigen. The results support the hypothesis that patients with autoimmune sensorineural hearing loss produce autoantibodies to an inner-ear supporting cell antigen that is phylogenetically conserved and defined by the murine monoclonal antibody KHRI-3. Since KHRI-3 can induce hearing loss after infusion into the inner ear, it is likely that autoantibodies with the same antigenic target are also pathogenic in humans.

CME program. Tracheotomy and inhalation injury.

Indications for tracheotomy in patients with respiratory burns have been unclear. Much of the literature is inconclusive or misleading. A thorough understanding of the pathogenesis, diagnosis, and therapy of inhalation injury is necessary to understand the role of tracheotomy. A critical review reveals relatively clearcut indications for the use of tracheotomy in inhalation injury, but many unanswered questions. Increased participation by otolaryngologists in burn management is recommended.

Computed tomography of the face and paranasal sinuses: Part I. Normal anatomy.

Computed tomographic (CT) scanning of the face and sinuses has improved markedly with modern equipment and methods. Expertise in facial and sinus anatomy as seen by CT is invaluable to the clinician. A review of normal CT anatomy is presented as a basis for diagnosing pathologic conditions.

Computed tomography of the face and paranasal sinuses: Part II. Abnormal anatomy and pathologic conditions.

Computerized tomography is extremely useful in delineating abnormal anatomy and pathologic conditions of the face and paranasal sinuses. The familiarity with normal anatomy is essential. Expert interpretation of CT scanning allows superior diagnosis of and treatment planning for lesions of the face and paranasal sinuses.

Voice disorders.

Many other diagnoses and treatments are of particular importance in caring for voice patients. Those discussed in this article are among the most common. The exacting demands of a professional singer or actor, his or her acute ability to analyze the body's condition, and his or her professional athlete's need for a nearly perfect result provide special challenges and gratification for physicians. However, a great many of our non-singer/actor patients are also extremely dependent on voice quality and endurance. Consequently, every patient with a voice complaint should be treated as Pavaroti or Sutherland would be treated, and every voice evaluation should be carried out systematically until a diagnosis has been made on the basis of positive evidence. The emergence of interdisciplinary teams, advanced instrumentation, and voice laboratories has made dramatic improvements in the standard of voice care, and the rapid development of voice as a subspecialty promises continuing advances in the care of voice disorders.

Surgical management of the frontal sinus.

The management of frontal sinus injury from trauma or operation has been controversial. Case reports and extensive literature review emphasize the importance of careful treatment of the frontal sinus whenever it is violated. The principles of management are reviewed.

Embryology of the facial nerve and its clinical applications.

Although many studies have investigated facial nerve development, most have been limited by available specimens and have not provided a comprehensive description of the development of this important structure. Even the best and most comprehensive articles on this subject have not systematically integrated the descriptions of facial nerve embryology with a summary of the development of other otologic structures. This study describes the parallel development of the facial nerve and ear, emphasizing practical applications for the otologic surgeon. The validity of these predictions has been tested through surgical procedures on patients with malformed ears.

Arytenoid dislocation: diagnosis and treatment.

Disruption of the cricoarytenoid joint is a relatively uncommon event, according to the world literature. Only 31 reported cases of arytenoid dislocation or subluxation exist other than the 26 cases described in this paper. Often cases are misdiagnosed as vocal fold paralysis. Knowledge of the signs and symptoms of arytenoid dislocation aids in correct diagnosis and early treatment. Even when diagnosis has been delayed, surgery may be highly effective. Familiarity with state-of-the-art diagnostic techniques and new concepts in management helps optimize the chances for good voice quality.

Lipoinjection for unilateral vocal cord paralysis.

Injection of Teflon paste is a commonly accepted procedure to improve the caliber of voice in unilateral vocal cord paralysis. There are several drawbacks to Teflon injection, among them respiratory obstruction (from overinjected Teflon) and unsatisfactory voice quality (Teflon causes stiffness of the vocal folds). This paper is a preliminary report on lipoinjection instead of Teflon injection into a paralyzed vocal fold. Fat appears to impart a soft bulkiness to the injected cord, while allowing it to retain its vibratory qualities. It is autologous material and can be retrieved if excessively overinjected. The fate of autologous fat injected into a paralyzed vocal cord remains unknown. Most of the literature on lipoinjection concerns repairs of depressed scars or breast augmentation. Our longest follow-up has been 12 months. Three patients have had this procedure, and the results appear to be very encouraging.

Male soprano voice: a rare complication of thyroidectomy.

A soprano voice from cricothyroid fusion is a rare complication following thyroidectomy. Thyroid surgeons should be aware of this possibility and recognize it early if voice pitch rises following thyroid surgery. This patient's unfortunate complication may prove fortuitous for phonosurgeons and their patients. Cricothyroid fusion may provide a better long-term retention of frequency elevation than traditional cricothyroid approximation. It is also a reversible procedure. Cricothyroid fusion should be investigated as an alternative to cricothyroid approximation for pitch modification.

Total en bloc resection of the temporal bone and carotid artery for malignant tumors of the ear and temporal bone.

A technique for single stage total en bloc resection of the temporal bone and intratemporal carotid artery with immediate reconstruction has been described. This formidable procedure requires the collaborative efforts of neurotologic skull base surgeons, neurosurgeons, and head and neck surgeons. Two patients have undergone this procedure; one with squamous cell carcinoma and one with extensive basal cell carcinoma. The quality of life following this procedure is adequate and the cosmetic deformity can be minimized. Longer follow-up is necessary to determine the value and overall impact of this approach to cancer of the temporal bone on disease free interval and patient survival. Further experience with the technical aspects of this procedure should reduce the operating time, blood loss, and resultant morbidity.

Second malignant neoplasms in patients under 40 years of age with laryngeal cancer.

OBJECTIVES/HYPOTHESIS: To determine the incidence of second malignant neoplasms (SMN) in patients under 40 years of age with invasive squamous cell carcinoma (SCC) of the larynx. STUDY DESIGN: Retrospective. METHODS: Using a National Cancer Institute tumor registry database encompassing 1973-1996, the incidence of SMN in patients under 40 years of age with laryngeal cancer was determined and compared with that of the registry's older, more traditional laryngeal cancer population. Median follow-up was 136 months. RESULTS: Among the 364 patients under the age of 40 years with laryngeal cancer, 30 (8.2%) had developed a secondary malignancy to date. In comparison, 4876 (21.4%) of 22,786 patients 40 years or older with laryngeal cancer were affected by an SMN. Kaplan-Meier analysis of the younger cohort projected 3.0%, 6.8%, and 10.7% relative risk of developing a SMN at any site over 5-, 10-, and 15-year periods, respectively, after index tumor diagnosis. Similar results for the older cohort were 14.2%, 28.1%, and 39.4% at 5, 10, and 15 years, respectively. Further Kaplan-Meier analysis demonstrated at least a fourfold increased risk for the development of secondary upper aerodigestive tract malignancies among older compared with younger patients. CONCLUSION: Patients under 40 years of age with invasive SCC of the larynx are significantly less likely to develop a second malignancy than their older counterparts.

Antibodies to HSP-70 in normal donors and autoimmune hearing loss patients.

OBJECTIVE: To evaluate serum antibody to heat shock protein (HSP) 70 as a marker for autoimmune sensorineural hearing loss (AISNHL). DESIGN: Sera from 20 patients with rapidly progressive sensorineural HL and 20 control volunteers without HL were tested for antibody reactivity against multiple HSP 70 substrates. Substrates included recombinant human HSP (rHuHSP) 72, purified bovine brain heat shock cognate (HSC) 73 and HSP 72, as well as heat-shocked and non-heat-shocked protein extracts from bovine kidney (MDBK) cells. All serum donors were previously tested for antibody to guinea pig inner ear supporting cells; 17 of 20 patients but none (0 of 20) of the controls were positive. METHODS: Sera were tested using Western blots. RESULTS: Reactivity with rHuHSP 70 was observed in 16 patients and 17 controls. Similarly, 15 of 20 patients and 17 of 20 controls stained for both HSP 72 and HSC 73 from the bovine brain. When tested against the heat-shock-induced and control MDBK extracts, six patients and nine controls had greater reactivity with the induced HSP 72. CONCLUSION: The frequency of antibodies to HSP substrates did not differ in patients and controls. Prior studies reported that HSP 72 is the 68 kD antigen commonly detected by AISNHL sera. However, we show that HSP 72 antibodies are no more prevalent in patients than in normal controls. Thus, it is unlikely that the 68 kD protein is HSP 72. Therefore, HSPs are not appropriate substrates for serodiagnosis of AISNHL.

Frequency of cochlear enhancement on magnetic resonance imaging in patients with autoimmune sensorineural hearing loss.

OBJECTIVE: To evaluate magnetic resonance imaging (MRI) scans for enhancement of inner ear structures of patients with sensorineural hearing loss and documented antibodies to the 68-kd inner ear antigen. STUDY DESIGN: Retrospective case review with reexamination of MRI scans. SETTING: Outpatient office. PATIENTS: Thirty-five patients with autoimmune sensorineural hearing loss defined by audiograms documenting a sensorineural hearing deficit in one or both ears and the presence of an anti-inner ear antibody (68-kd band) in serum samples who underwent precontrast and postcontrast T1-weighted axial and coronal MRI scans of the inner ear, which were performed concurrently with the hearing loss. INTERVENTIONS: Diagnostic. MAIN OUTCOME MEASURES: Frequency and intensity of cochlear enhancement on MRI scans. RESULTS: One patient demonstrated +2 cochlear enhancement. However, that finding was thought to represent postoperative inflammatory change. CONCLUSION: No correlation was found between the presence of antibodies to inner ear antigen in patients with hearing loss and cochlear enhancement on MRI scans.

Autologous fibrin tissue adhesive for peripheral nerve anastomosis.

The sciatic nerve in rabbits was exposed, transected, and repaired using either of two methods. The experimental group had an autologous fibrin-based adhesive repair. The control group had conventional perineural suturing. The autologous tissue glue is similar in composition, handling, and performance to the commercially available European product Tisseel. It can be prepared quickly without sophisticated equipment or expense. Autologous glue obviates the risk of transmissible disease that has precluded approval of the commercial product for use in the United States. The glued anastomosis can be performed with accurate apposition and minimal trauma to the nerve. By making a fibrin cocoon, the anastomosis can be insulated. This provides some of the benefits of sheathed anastomosis while minimizing foreign body reaction. Functional evaluation and histologic results comparing the amount of anastomotic fibrosis, axonal regeneration, and alignment of fascicles showed glue to be as good as, if not superior to, conventional suture technique.

Bilateral synchronous mixed tumors of the parotid glands.

Bilateral synchronous mixed tumors of the parotid glands are uncommon. In this case, they occurred in association with multiple benign tumors elsewhere in the body. The larger of the two parotid neoplasms was asymptomatic and undetected on physical examination, even after its presence had been established by computed tomographic scan.

Compatibility of autologous fibrin adhesive with implant materials.

A safe, effective method for securing autologous and alloplastic implants would benefit modern surgery substantially. We studied the effects of a new autologous fibrin tissue adhesive with various alloplastic implants and ear cartilage. Twelve rabbits had Proplast (a woven combination of Teflon and organic fibers), Silastic, Supramid polyfilament surgical sheeting, and ear cartilage implanted. One side of each animal had implants secured with tissue adhesive. The other, control side had identical implants placed in "subcutaneous tunnels." The tissue adhesive was biodegradable and produced no apparent toxic reaction, infection, or foreign-body reaction. It did not adhere to Silastic, but bound to the other implants. Glued implants had no more clinical or histologic evidence of inflammation than controls. Autologous fibrin tissue adhesive seems to be promising as a safe, biologic bonding material for securing certain alloplastic and autologous implants.

Cornelia de Lange syndrome. Otolaryngologic manifestations.

Cornelia de Lange syndrome (CDLS) is characterized by multiple congenital malformations and mental retardation. New studies have documented otolaryngologic abnormalities. We examined 45 patients with CDLS. Virtually all had hearing loss, and most had impaired language development. Other otolaryngologic abnormalities, including external auditory canal stenosis and cleft palate, were also common. Because of the high incidence of otolaryngologic abnormalities, and the need for early intervention, it is important for otolaryngologists to recognize CDLS manifestations in the head and neck.

The sensitivity of auditory brainstem response testing for the diagnosis of acoustic neuromas.

OBJECTIVES: To determine the sensitivity of auditory brainstem response (ABR) testing for detecting acoustic neuromas and to determine whether the test is less sensitive for detecting small tumors. DESIGN: Retrospective review of the medical charts of 58 patients with acoustic neuroma who had all of the data necessary for inclusion in the study. SETTING: University-affiliated referral practice of one neurotologist. PATIENTS: Patients with acoustic neuromas who had both ABR tracings and magnetic resonance imaging films available for review. MAIN OUTCOME MEASURES: Positive ABR and negative ABR results correlated with tumor size. RESULTS: Tumor size ranged from 0.4 to 7 cm. The overall sensitivity of ABR in diagnosing acoustic neuromas was 90%. However, ABR was progressively less sensitive with decreasing tumor size. Only 7 (58%) of the 12 tumors 1 cm or smaller were detected by ABR. CONCLUSION: Auditory brainstem response testing cannot be relied on for detection of small acoustic neuromas and should not be used as a criterion to determine whether magnetic resonance imaging should be performed when an acoustic neuroma is suspected clinically.