Hearing disability and hearing aid benefit related to type of hearing impairment

This study was designed to investigate whether patients with a conductive hearing impairment derive more benefit from the provision of a hearing aid than comparable individuals with a sensorineural hearing impairment. A secondary aim was to assess the relative hearing disability of those with each type of impairment. Twenty-eight patients with a bilateral, symmetrical conductive impairment were selected. They were matched for age, sex and speech frequency average to individuals with a bilateral, symmetrical sensorineural hearing impairment. Each patient performed free-field audio and audio-visual tests in noise, both with and without a hearing aid, during which the non-test ear was acoustically occluded. When unaided, individuals with a conductive impairment were more disabled than those with a sensorineural impairment. On the other hand, those with a conductive impairment derived more benefit from an aid than those with a sensorineural impairment.     

The Baha Softband. A new treatment for young children with bilateral congenital aural atresia

The Baha (bone-anchored hearing aid) Softband appears to be an effective mean of hearing rehabilitation for children with a congenital bilateral aural atresia who are too young for the amplification of a Baha on an implant. The aided hearing threshold with a Baha Softband is almost equal to that achieved with a conventional bone conductor. The speech development of the children studied with a Baha Softband is on a par with peers with good hearing.     

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).     

Phonological representations in children with SLI: a study of French.

The present research examined the quality of the phonological representations of French children with specific language impairment (SLI) and those with normal language development (NLD). Twenty-five children with SLI and 50 children with NLD matched on lexical age level participated in an auditory lexical decision task. The observations gathered in our study can be summarized as follows. First, children with a higher receptive lexical level performed better, and this was true both for children with NLD and children with SLI. Second, both children with NLD and those with SLI were more likely to reject pseudowords resulting from a modification affecting the number of syllables of a word than pseudowords resulting from a slight modification with the number of syllables unchanged. This difference, however, was greater for the children with SLI, who appeared to have much difficulty rejecting pseudowords resulting from slight modifications. Finally, the performance of children with SLI was particularly poor when presented with pseudowords resulting from a slight modification at the beginning or the end of a word. These findings are interpreted as supporting the hypothesis of an under-specification of phonological representations in children with SLI.     

Echinococcosis presenting as an otogenic brain abscess: an unusual lesion of the middle ear cleft and temporal lobe

This paper presents a case of a 28-year-old male with a seizure episode and a 4-year history of intermittent tinnitus on the left ear. On computed tomography and magnetic resonance imaging, a density with rim enhancement was found at the temporal lobe, associated with mastoid tegmen destruction and middle ear mass, indicating cholesteatoma with complicating brain abscess. Evacuation of the brain abscess was performed with a combined otolaryngologic and neurosurgical procedures (canal wall-down mastoidectomy and temporal craniotomy). The pathology turned out to be infestation with Echinococcus granulosus.     

Comparison of functional and quality-of-life outcomes in patients with and without palatomaxillary reconstruction: a preliminary report

BACKGROUND: Orodental rehabilitation of hemipalatomaxillectomy defects can be accomplished by using a prosthetic obturator or a vascularized bone-containing free flap. Whereas prosthetic obturation offers several advantages, including the opportunity for immediate dental restoration without the need for further surgery, vascularized bone grafts provide permanent closure of the oronasal communication and bone sufficient for the placement of osseointegrated implants. OBJECTIVE: To compare the functional and quality-of-life (QOL) outcomes in patients rehabilitated with a prosthetic obturator with defect-matched patients who underwent reconstruction with a vascularized bone-containing free flap. METHODS: Four hemipalatomaxillectomy patients rehabilitated with a tissue-borne prosthetic obturator were compared with 4 defect-matched hemipalatomaxillectomy patients who underwent reconstruction with a vascularized bone-containing free flap. All of the patients were objectively assessed for speech, mastication, and QOL. Functional status was assessed by mastication testing, voice analysis, and nasorhinometry. Swallowing-related QOL was assessed using a patient-reported, validated swallowing QOL questionnaire, and donor site morbidity was assessed using upper extremity and lower extremity questionnaires. RESULTS: Patients who underwent reconstruction with a vascularized bone-containing free flap achieved higher mastication and speech assessment scores with less oronasal reflux than defect-matched patients rehabilitated with a prosthetic obturator. Swallowing QOL and donor site assessments demonstrated that compared with their prosthetic counterparts, reconstruction patients enjoyed a better QOL without incurring significant donor site morbidity. CONCLUSIONS: Although palatomaxillary reconstruction with vascularized bone-containing free flaps requires a second operative site, this method of orodental rehabilitation of the hemipalatomaxillectomy defect can achieve superior functional and QOL outcomes relative to defect-matched patients rehabilitated with a prosthetic obturator.     

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.     

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.     

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.     

An 8-year-old boy with a Pott's puffy tumor

An 8-year old boy with a history of trauma, sinusitis and a swelling of the frontal bone with somnolence was diagnosed with a Pott's puffy tumor (PPT). Minimal invasive surgical intervention was performed together with a strict regimen of antibiotic therapy. In this case debridement of the frontal bone was not necessary. Serial X-ray imaging of the skull showed complete ossification of the frontal bone lesion. Early diagnosis using thorough radiological evaluation is necessary to effectuate the proper therapeutic approach. For this reason, a patient with a forehead swelling and a history of trauma and/or sinusitis should be suspected for a PPT.     

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.     

A case of barotrauma-induced pneumolabyrinth secondary to perilymphatic fistula

We report the case of a 62-year-old woman who experienced pneumolabyrinth associated with a perilymphatic fistula. Her condition was diagnosed with the help of computed tomography, which detected the presence of an air bubble in the labyrinth, and middle ear exploration, which revealed that clear fluid was emanating from the round window niche in a manner consistent with the presence of a perilymphatic fistula. The niche was repaired with tragal perichondrium and bolstered with Gelfoam.     

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.     

Cystic hamartoma of the mandibular angle. Apropos of a case

A large cyst of mandibular angle was surgically removed in a 20 years-old woman. This cyst was discovered during childhood and was associated with a branchial para-mandibular cyst. It was histologically lined either with a malpighian epithelium with sebaceous islands or with a ciliated cylindrical epithelium. Because of its topography, its association with a branchial cyst and its histological feature quite similar to that of a cyst of thyroglossal duct, this cyst was thought to represent a simplified salivary ectopy composed only of a cystic secretory duct without any salivary parenchyma.     

Diagnosis of nasopharyngeal carcinoma metastatic to mediastinal lymph nodes by endoscopic ultrasonography-guided fine-needle aspiration biopsy

We report the case of a patient with nasopharyngeal carcinoma who was diagnosed as having metastasis in mediastinal lymph nodes and successfully underwent systemic chemotherapy without surgery. A 61-year-old male with a history of nasopharyngeal carcinoma presented with odynophagia. Examination revealed two palpable lymph nodes in the right neck. Pharyngoscopy showed a mass in the left inferior pharyngeal mucosa, and upper gastrointestinal endoscopy showed only chronic gastritis, with no sign of esophageal disease. Chest CT confirmed the presence of a non-enhancing 20-mm soft tissue mass in the paraesophageal area, with increased attenuation compared with the adjacent esophagus. To evaluate this lesion we applied endoscopic ultrasonography-guided fine-needle aspiration biopsy (EUS-FNA). Two passes were made with a 21-gauge fine needle and the patient tolerated the procedure well, without complications. Cytological findings were compatible with metastatic squamous cell carcinoma from a nasopharyngeal tumor, and the clinical stage was determined as T3N2bM1 (stage IVC) because of mediastinal lymph node metastasis. We thus determined the nodal status of a head and neck tumor by means of EUS-FNA. In conclusion, EUS-FNA is a safe and reliable technique for evaluation of mediastinal lymphadenopathy, and is especially valuable for head and neck tumors with suspected metastasis.     

Surgical rehabilitation of neuromuscular swallowing disorders with special regard to cricopharyngeal myotomy and glottopexy]

Surgical Rehabilitation of Neuromuscular Swallowing Disorders with Special Regard to Cricopharyngeal Myotomy and Glottopexy. BACKGROUND: The surgical rehabilitation of patients with swallowing disorders caused by neuromuscular insufficiency with life-threatening aspiration presents a special challenge to the ENT-surgeon. METHODS: In a period of 5 years we decided on a surgical treatment in altogether 12 patients with paralytical dysphagia. In 6 patients we combined a cricopharyngeal myotomy with a complete closure of the glottis, in 5 patients we performed a sole cricopharyngeal myotomy. In another patient we restricted ourselves to glottopexy only. RESULTS: In all cases the dysphagia giving rise to the surgical intervention was regredient so far that the removal of the percutan endoscopic gastrostomy postsurgically was possible. 3 special cases are presented in detail. DISCUSSION: The main part of the therapy is the subtle and complete cricopharyngeal myotomy. Particulary good results are available with a combination of the latter with a reversible glottopexy. CONCLUSIONS: After the failure of conservative therapy the indication for a surgical treatment should be made on a large scale.     

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.     

Squamous cell carcinoma of the nasal vestibule

Squamous cell carcinoma of the nasal vestibule is a rare disease. Most advocate radiotherapy as a primary treatment for early tumours, with surgery reserved for salvage. For advanced disease, combined therapy with surgery and postoperative radiotherapy is generally recommended. Fourteen patients with squamous cell carcinoma of the nasal vestibule were reviewed. A classification of early versus late lesions was used. We achieved a 78% local regional control rate (minimum follow-up 3 years) in patients with early disease, with either radiotherapy or surgery as a primary modality of treatment. All patients with late disease recurred, requiring further surgical and/or radiation treatment. Only 20% of these patients were disease free at 2 years. Recurrent disease in either group, whether local or regional, carried a grave prognosis, with a 25% disease-free survival at 3 years.     

Cancer of the laryngeal vestibule. A retrospective study of 161 cases

We report a retrospective study of 161 cases of vestibular or epilaryngeal cancer treated by horizontal laryngectomy. The different factors affecting prognosis were analyzed statistically. Three groups were distinguished according to the size and primary site of the tumor: (1) a group with a very poor prognosis, which included patients with large tumors of the epilarynx; (2) a group with an average prognosis, which included small tumors of the epilarynx and large tumors with their primary site in the vestibule; (3) a group with a favorable prognosis which included tumors classified as T1 or T2 that were initially located in the vestibule.