Pitfalls in neurotologic diagnosis

Several cases illustrating potential pitfalls in neurotologic diagnosis from the viewpoint of the otologist are presented. The role of auditory brain stem response testing is specifically emphasized in cases with discordant audiologic and radiographic findings. Included are one case of a "false negative" ABR in a patient with an intracanalicular acoustic neuroma, a case of a "false positive" CT scan in a patient with Meniere's disease, and a case of a patient with normal hearing in whom an acoustic neuroma was discovered serendipitously.     

Perennial rhinitis: an analysis of skin testing, serum IgE, and blood and smear eosinophilia in 201 patients

Allergen skin testing, eosinophil counting in blood and nasal smear and serum IgE determination were performed in patients with simple perennial rhinitis, and in patients with rhinitis and asthma. The results were compared with those obtained in normal controls. As a positive skin test was demonstrated in 40% of patients with simple rhinitis and in 88% of patients with rhinitis and asthma, skin testing is considered relevant in all patients with perennial rhinitis. While blood eosinophil count and serum IgE level were elevated in a fair proportion of asthmatic patients, only a few subjects with simple perennial rhinitis had an elevated level, and the diagnostic values of these tests is questionable. On the other hand, examination of a nasal smear for eosinophils could usually separate the patients from the controls. The study emphasizes the importance of making a distinction between simple perennial rhinitis and perennial rhinitis associated with other symptoms and diseases.     

Results in terms of hearing results after surgical closure of labyrinthine fistulas

OBJECTIVES: We investigated the results achieved in terms of hearing in 36 patients who had each undergone resection of a cholesteatoma and closure of a labyrinthine fistula. PATIENTS AND METHODS: These patients included 28 who had a fistula of the semicircular canal, in 7 of them with an opened perilymphatic space. In 8 cases we found a fistula of the cochlea, with with a perilymphatic leak 7 in of them. We compared the sensorineural threshold before and after surgery and also the results in the two groups. Patients who were deaf preoperatively were excluded from further analysis. RESULTS: Hearing did not improve in any of the patients (n=5) who were deaf preoperatively. There was no significant difference in changes to the hearing threshold between patients with fistula of the cochlear and those with fistula of the semicircular canal with opened perilymphatic space; nor did patients with a fistula of the semicircular canal without perilymphatic leakage have a better outcome than patients with an open membranous labyrinth. CONCLUSIONS: The outcome of cholesteatoma resection and closure of the fistula in a single operation was good. Perilymphatic leakage seems to be predictive of a poorer result in terms of hearing.     

The effect of superior canal dehiscence on cochlear potential in response to air-conducted stimuli in chinchilla

A superior semicircular canal dehiscence (SCD) is a break or hole in the bony wall of the superior semicircular canal. Patients with SCD syndrome present with a variety of symptoms: some with vestibular symptoms, others with auditory symptoms (including low-frequency conductive hearing loss) and yet others with both. We are interested in whether or not mechanically altering the superior canal by introducing a dehiscence is sufficient to cause the low-frequency conductive hearing loss associated with SCD syndrome. We evaluated the effect of a surgically introduced dehiscence on auditory responses to air-conducted (AC) stimuli in 11 chinchilla ears. Cochlear potential (CP) was recorded at the round-window before and after a dehiscence was introduced. In each ear, a decrease in CP in response to low frequency (<2 kHz) sound stimuli was observed after the introduction of the dehiscence. The dehiscence was then patched with cyanoacrylate glue leading to a reversal of the dehiscence-induced changes in CP. The reversible decrease in auditory sensitivity observed in chinchilla is consistent with the elevated AC thresholds observed in patients with SCD. According to the ‘third-window’ hypothesis the SCD shunts sound-induced stapes velocity away from the cochlea, resulting in decreased auditory sensitivity to AC sounds. The data collected in this study are consistent with predictions of this hypothesis.     

Ventilation tubes and cochlear implants: what do we do?

OBJECTIVE: To determine current practice management with respect to ventilation tubes and cochlear implants. STUDY DESIGN: Questionnaire. SETTING: All members of the American Neurotology Society were sent questionnaires. MAIN OUTCOME MEASURES: Members were asked a series of questions including how they deal with ventilation tubes before cochlear implantation, how they manage serous otitis media in patients undergoing cochlear implantation, and how they manage otitis prone children with cochlear implants. RESULTS: Two hundred and twenty members returned questionnaires. Surgeons who replied perform an average of 25 implants per year: 15 in adults and 10 in children. Analysis of the data revealed a wide practice variation between surgeons. Fifty-six percent of surgeons will place a cochlear implant in a patient with a clean, dry ventilation tube in place. More than half the surgeons will place a ventilation tube in a child with serous otitis media, let the ear settle down, and perform the cochlear implant at a second operation. Wide variation in the management of otitis prone children with cochlear implants exists with respect to placement of ventilation tubes. Only 5% of surgeons reported any complications with cochlear implants that they attributed to ventilation tubes. There were a number who suggested their practice had changed since the recent identification of issues involving meningitis in implantees. CONCLUSION: Wide practice variation exists with the management of ventilation tubes in cochlear implant patients. On the basis of the results of this survey, it is acceptable to place cochlear implants in patients with clean, dry ventilation tubes. It also acceptable to place ventilation tubes in otitis prone children with cochlear implants. Despite theoretic concerns, the reported incidence of complications is low.     

The role of a family history in King Kopetzky Syndrome (obscure auditory dysfunction)

King Kopetzky Syndrome (KKS) is a common condition in which individuals with normal audiograms complain of hearing difficulties, particularly in noisy places. Several studies have shown many patients with KKS to have a family history of hearing problems. In 82 consecutive patients with KKS and normal middle ear function, we compared the performance of those with and without a family history of hearing impairment on a number of sensitized tests. Those with a family history were more likely to have notches on Audioscan testing (p < 0.005) and these notches were broader than those found in patients with no family history (p < 0.05). There was also a tendency for those with a family history to be more likely to have notches on DPOAEs (p < 0.07), and the reproducibility of the TOAEs was poorer in those with a family history. Psychological testing showed males with a family history to have higher scores on free-floating anxiety (p < 0.01) and obsessionality (p < 0.05).     

Eustachian tube function in patients with eosinophilic otitis media associated with bronchial asthma evaluated by sonotubometry

OBJECTIVE: To determine eustachian tube function in patients with asthma and with or without eosinophilic otitis media (EOM), a new middle ear disease entity with a highly viscous middle ear effusion containing many eosinophils and usually associated with bronchial asthma. One of the most important causes of otitis media (OM) is eustachian tube dysfunction. DESIGN: Retrospective case review. SETTING: A referral center. PATIENTS: Twenty patients with EOM and patients with asthma but without OM. MAIN OUTCOME MEASURES: We studied eustachian tube function using sonotubometry and a questionnaire. Sonotubometry was also performed on 13 control patients with chronic otitis media (COM) and 7 normal controls. RESULTS: The tubal opening duration was significantly longer in patients with EOM than in patients with asthma but without OM, controls with COM, and normal controls, indicating the presence of patulous eustachian tubes in patients with EOM. Responses to the questionnaire also supported the presence of patulous eustachian tubes in the patients with EOM. CONCLUSIONS: The presence of a patulous eustachian tube may be a major cause of EOM in patients with bronchial asthma. In patients with asthma who have a helper T-cell 2-dominant predisposition, a patulous eustachian tube easily allows the entry of antigenic materials into the middle ear, causing eosinophil-dominant inflammation.     

Otitis media with effusion in the adult

The etiologic factors associated with serous otitis media or otitis media with effusion (OME) are well described in children. Little has been published with regard to OME and its etiology in adults. Thirty-five adult patients who presented with OME in a referral-based otolaryngologic practice over a five-year period were studied by retrospective chart review with a follow-up telephone interview. OME in adults may be related to previous middle ear surgery in the contralateral ear, a history of middle ear disease in childhood, chronic nasal symptoms of obstruction and nasal fullness, cigarette smoking, and a family history of middle ear disease. The onset is often associated with upper respiratory tract infection. Nasopharyngeal carcinoma was uncommon.     

The long-term results of the treatment with transtympanic ventilation tubes in children with chronic secretory otitis media

In the present report we discuss the short-term and long-term results of treatment with transtympanic ventilation tubes in 140 children with chronic secretory otitis media. Important findings are: after 6 months only 40% and after 12 months only 5% of the inserted grommets are still draining. A good clinical result is obtained in 72% after 6 months, and in 52% after 12 months. With a long follow-up, up to 10 years, 71% of the children suffering from chronic secretory otitis media and treated with grommets, can be considered as cured, with or without a small complication. The poorest results are obtained after a follow-up of 2-3 years. Children under 7 years have a better prognosis than older children. There is also a better prognosis in children with serous effusions than in those with a glue ear. We noticed very few complications in our treatment with grommets: no sensorineural hearing loss and a persisting perforation in 2% of the treated ears.     

A foreign body embedded in the mobile tongue masquerading as a neoplasm

Foreign bodies in a mobile tongue are rarely presented to the laryngologist, because such bodies are commonly lodged superficially and are easily removed by the patients themselves or by general practitioners. Thus, it is rare that a foreign body totally embedded in the mobile part of the tongue presents as an enlarged tongue mass. We have described a 64-year-old female with a 3-month history of an enlarged mass in the anterior right tongue. Physical examination showed a mass located in the anterior right tongue, with intact mucosa and normal color. A benign tongue neoplasm was considered first. However, a fish bone totally embedded in the mobile tongue with granuloma formation was encountered during the incisional biopsy operation. Complete removal of the foreign body with granuloma was achieved under local anesthesia. There was no neuromuscular or neurosensory deficit of the tongue in the follow-up period of 2 years. Although an embedded foreign body in the mobile tongue is a rare condition, it should be considered in the work-up of a patient with an enlarged tongue mass, with or without a history of swallowing a foreign body.     

Relationship of biopsy and final specimens in evaluation of tumor thickness in floor of mouth carcinoma

A number of studies have suggested that tumor thickness may be a valuable prognostic indicator in the evaluation of head and neck cancers. This study examined the relationship between tumor thickness measured in preliminary biopsy specimens with the final specimens obtained in 31 patients patients with floor of mouth epidermoid carcinoma. There was a significant statistical correlation between biopsy and final specimens. The Pearson's product-moment correlation coefficient was 0.58, which corresponded to a significance level of P<.0005. The results of this study showed that those patients who had biopsies with a thickness less than or equal to 1 mm were likely to have final specimens with a thickness less than 2 mm. All patients with a thickness greater than 2 mm had a final specimen with a thickness greater than 3.5 mm. Modification of current biopsy techniques may result in values more predictive of final thickness measurements.     

Effect of bimodal hearing in Korean children with profound hearing loss

Conclusion. Bimodal hearing with combined acoustic stimulation and electric stimulation could enhance speech performance in deaf patients by residual hearing even though the amount of residual hearing is not enough to be used for communication by amplification. Objectives. The cochlear implant (CI) is a well-known therapeutic option for patients with profound hearing loss. However, deaf patients with a CI still have trouble in localization of sounds and understanding speech in a noisy environment. The aim of this study was to evaluate the benefits of bimodal hearing with a CI in one ear and a hearing aid in the contralateral ear in Korean children with profound hearing loss. Subjects and methods. Fourteen deaf children with residual hearing participated in this study. There were eight male and six female patients, with an age range of 4.6–13.8 years at the time of testing. The test was conducted between 3 months and 4.2 years after cochlear implantation. Speech performance was examined in a noisy environment using Korean word lists. A speech sound and the noise were presented to the child from the front loudspeaker. Results. The results showed that speech performance in a noisy environment was significantly better with bimodal hearing than with a CI alone.     

Chemotactic properties of retained maxillary sinus secretions

The chemotactic activity of 82 aspirated maxillary sinus secretions obtained from 32 sinuses in 29 patients was assayed with a modified Boyden chamber technique. The secretions were also analysed with respect to the proteolytic activity according to a modification of a technique described by Moroz. In only 3 of 24 sinus secretions obtained from untreated patients with purulent or mucopurulent sinusitis, but in 5 of 8 serous secretions from untreated patients with serous sinusitis a chemotactic activity exceeding random migration was found. A high proteolytic activity was found to be incompatible with a high chemotactic activity. Regarding mucopurulent and purulent sinusitis, treatment by repeated antral aspiration resulted in an increase of the proportion of chemotactically active secretions and a decrease of the proteolytic activity. Repeated antral aspirations in patients with serous sinusitis resulted in less uniform changes of the chemotactic activity.     

Oral cavity and oropharyngeal tumors in human immunodeficiency virus-positive patients: acute response to radiation therapy.

BACKGROUND: The survival of patients with human immunodeficiency virus (HIV) has improved considerably with modern medical management. However, there remains surprisingly little information on treating head and neck neoplasms in HIV-positive patients. OBJECTIVE: To report our recent experience treating oral cavity and oropharyngeal tumors in HIV-positive patients. DESIGN AND PATIENTS: Retrospective analysis of a cohort of 8 HIV-positive patients with Kaposi sarcoma (KS), lymphoma, or squamous carcinoma of the oral cavity or oropharynx who were consecutively treated during a single year with radiation therapy at a tertiary care referral center. Length of follow-up was at least 2 years (mean, 2.5 years). RESULTS: All patients had partial and complete responses to treatment lasting until the last follow-up. However, we found that treatment was considerably better tolerated by patients with non-KS tumors, with fewer acute reactions and significantly less weight loss, despite larger treatment volumes and higher radiation doses, compared with patients with KS. Patients with non-KS tumors received a mean radiation dose of 62.6 Gy to 2636 cm3, yet lost only a mean of 0.1 kg in weight, whereas patients with KS were treated with a mean radiation dose of 19 Gy to a mean volume of 568 cm3, but lost a mean of 5.8 kg during treatment (P = .005) and on average sustained an additional grade of severity on a standard scale of mucosal reaction (P = .01). CONCLUSIONS: Oral cavity and oropharyngeal tumors in HIV-positive patients respond to radiation therapy, but there is a marked difference in the degree of acute reactions to treatment between patients with and without KS. Infection with HIV is not a contraindication when aggressive radiation therapy is needed in select patients.     

Tympanometry in the detection of hearing impairments associated with otitis media with effusion

The monitoring of children with otitis media with effusion ties up considerable resources in audiology departments. Impedance audiometry is frequently used when investigating these children. It has been shown to be highly sensitive in detecting middle ear effusion, but its value in identifying those children with a significant hearing impairment secondary to this is in question because of the wide range of hearing impairments possible with a type B tympanogram. This study quantified the sensitivity and specificity of impedance audiometry in detecting a hearing impairment greater than or equal to 25 dB HL due to otitis media with effusion. The subjects were 285 children of whom 20% had hearing thresholds greater than or equal to 25 dB HL. A peaked tympanogram (types A or C) virtually eliminated the possibility (98% confidence) of such a hearing impairment. A flat type B tympanogram was satisfactorily sensitive (93%) in detecting a hearing impairment, but non-specific (76%). The positive predictive value was 49%, i.e. 51% of ears with this type of tympanogram had hearing within acceptable limits. Assuming that a sensorineural impairment has been excluded and a population is being monitored for hearing impairment associated with otitis media with effusion, it is suggested that the presence of a peaked tympanogram indicates normal hearing, whereas those children with a flat tympanogram would require their hearing to be evaluated. Increased use of impedance audiometry to monitor children with otitis media with effusion would reduce the number requiring full pure-tone audiometry with a subsequent reduction in the workload of an audiology department.     

Prognosis and therapy of early acute idiopathic auditory failure

The prognostic value of some otovestibular parameters was evaluated in 46 cases of acute idiopathic hypoacusis (AIH) and 12 cases of acute idiopathic anacusis (AIA) observed within 7 days of the onset. Recovery in AIH is predicted by some otoneurological parameters as mean pure-tone hearing threshold (PTA) for 500, 1,000 and 2,000 Hz, stapedial reflex and severe vertigo. None of these measures alone seems to have prognostic value. The expected recovery rate also is reflected in the various parameters taken as a whole. Subjects with mean PTA better than 70 dB HL together with stapedius reflex present for 500 and 1,000 Hz, no decay and no vertigo have a very favourable prognosis: 86% had complete or good recovery. On the contrary, subjects with mean PTA worse than 70 dB HL and with pathological stapedius reflex have a generally unfavourable prognosis: 35% had complete or good recovery. Vertigo as a symptom does not seem in itself to have an unfavourable prognostic value. Actually, patients with severe vertigo generally have a poor recovery. Vertigo as a symptom indeed is frequently associated with a more severe sensorineural lesion. Carbogen or heparin-dextran are not an effective treatment: complete-good global recoveries obtained in AIH without any treatment, with carbogen or with heparin-dextran were, respectively, 65, 68 and 62%. As a consequence, an early therapy with these substances has no apparent value. Patients affected with AIA have a very unfavourable prognosis. All of our patients with AIA had poor recovery. For the moment we feel it is easier to reach a satisfactory prognosis rather than to institute an effective or causal therapy in acute idiopathic auditory failure.     

Brain herniation and space-occupying lesions eroding the tegmen tympani

Technological advances in neuroradiology and the development of skull base surgery in neurotology have improved diagnosis and management of lesions eroding the tegmen tympani. The diagnosis of brain hernia is to be suspected in patients with a history of complicated chronic ear surgery and a slowly developing pulsatile mass with CSF leak. Patients are best evaluated in the upright position, with an otomicroscope and by magnetic resonance imaging (MRI). Over 6 years, our group has treated seven patients with eight space-occupying lesions eroding the tegmen. Five of the lesions were repaired with a temporalis muscle flap, 2 with fascia and bone, and 1 with Marlex. A review of new technology in the diagnosis of brain hernia and a modification of previous techniques is given.     

Pulsatile tinnitus and therapeutic embolization.

Fifteen patients with disabling pulse-synchronous tinnitus were investigated with super-selective angiography demonstrating an arteriovenous malformation in 8 cases, chemodectoma of the jugular bulb in 3, and a local arterial stenosis in one case. In 12 of these cases the murmur could be registered objectively, while in the 3 cases with a negative angiographic finding no such murmur could be heard, an observation which may be of importance when selecting patients for further angiographic examination. The cases with a tumour and those with an arteriovenous malformation were all treated with gelatin sponge embolizatioected cases embolization is recommended alone or in combination with surgery.     

Neurofibromatosis type 1, hyperparathyroidism, and osteosarcoma: interplay?

Neurofibromatosis type 1 (NF 1) is a syndrome with a predisposition for benign and malignant tumor development. Of the malignant neoplasms, osteogenic sarcomas are rare but have been described. There are some reports of patients with neurofibromatosis type 1 with a parathyroid adenoma and hyperparathyroidism. Also, there are studies that imply that the parathyroid hormone plays a role in the regulation and modulation of oseogenic sarcomas in vitro. We report about a 50-year-old female suffering from neurofibromatosis type 1, with a 3-year documented history of untreated hyperparathyroidism and a parathyroid adenoma. The patient developed a mandibular osteogenic sarcoma. To our knowledge, this is the first reported case occurring in the mandible. The unusual tumor site for a patient with neurofibromatosis type 1, the conjugation with hyperparathyroidism and the rapid growth of an osteogenic sarcoma are intriguing.     

Long-term audiological feature in Pendred syndrome caused by PDS mutation

Pendred syndrome is an autosomal recessive disorder characterized by profound deafness in childhood and goiter. We report a case of Pendred syndrome in a 27-year-old woman who had a diffuse goiter and progressive sensorineural hearing loss with fluctuation and a missense mutation (His723Arg) in the PDS gene identified in a homozygous state. Audiological findings were observed clinically over a 20-year period. Progressive hearing loss with fluctuation occurred before age 12 years. An enlarged vestibular aqueduct with enlargement of the endolymphatic duct and sac was confirmed with 3-dimensional magnetic resonance imaging hydrography.