Fulminant intracranial hypertension as a result of otological surgery: case report and discussion of management.

BACKGROUND: Dural venous sinus injury is a rare complication of otological surgery that can lead to life-threatening sequelae, the management of which is complex and poorly described. CASE REPORT: This paper describes the case of a 40-year-old female who underwent routine right myringoplasty complicated by sigmoid sinus laceration. The patient subsequently developed right-sided lateral sinus thrombosis leading to fulminant intracranial hypertension. The patient underwent successful emergency management by surgical reconstruction of the sigmoid sinus, followed by endovascular thrombolysis, catheter balloon angioplasty and endovascular stenting. CONCLUSION: Torrential haemorrhage following otological procedures is uncommon and rarely requires packing of a bleeding venous sinus. This case highlights that injury to a highly dominant venous sinus can lead to venous outflow obstruction and life-threatening intracranial hypertension. To our knowledge, the development of this complication following otological surgery and its management has not been reported previously.

Hearing outcomes of cochlear implant recipients with pre-operatively identified cochlear dead regions.

Objectives: To determine how patients who did not meet UK TA166 NICE criteria for cochlear implantation (CI) but were subsequently found to have cochlear dead regions (DRs) performed with CI.Methods: A retrospective review of medical records was performed on CI recipients: 152 controls and 40 in the DR group. Of these, 34 pairs were matched by pre-operative Bamford-Kowal-Bench (BKB) scores and compared.Results: The forty DR patients had a median age at implantation of 56 years. Their mean pre-operative BKB score of 23% increased to 78% after CI. Thirty-seven experienced improvements in BKB scores. In matched case-control analysis, the improvement in mean BKB score with CI was no different (p = 0.19) between the DR group and control group; a similar proportion of patients benefitted in each group.Discussion: This study is the largestreport to date of performance of patients with DRs, before and after CI. The DR group gain similar benefit as the controls.Conclusion: Patients with DRs, who did not meet TA166 NICE criteria, received the same benefit as those who did. TEN testing to detect DRs should be included in routine CI work-up where standard criteria are not met.

The Cambridge Otology Quality of Life Questionnaire: an otology-specific patient-recorded outcome measure. A paper describing the instrument design and a report of preliminary reliability and validity.

OBJECTIVE: The Cambridge Otology Quality of Life Questionnaire (COQOL) is a patient-recorded outcome measurement (PROM) designed to quantify the quality of life of patients attending otology clinics. DESIGN: Item-reduction model. A systematically designed long-form version (74 items) was tested with patient focus groups before being presented to adult otology patients (n. 137). Preliminary item analysis tested reliability, reducing the COQOL to 24 questions. This was then presented in conjunction with the SF-36 (V1) questionnaire to a total of 203 patients. Subsequently, these were re-presented at T + 3 months, and patients recorded whether they felt their condition had improved, deteriorated or remained the same. Non-responders were contacted by post. A correlation between COQOL scores and patient perception of change was examined to analyse content validity. SETTING: Teaching hospital and university psychology department. PARTICIPANTS: Adult patients attending otology clinics with a wide range of otological conditions. MAIN OUTCOME MEASUREMENTS: Item reliability measured by item­total correlation, internal consistency and test­ retest reliability. Validity measured by correlation between COQOL scores and patient-reported symptom change. RESULTS: Reliability: the COQOL showed excellent internal consistency at both initial presentation (a = 0.90) and 3 months later (a = 0.93). Validity: One-way analysis of variance showed a significant difference between groups reporting change and those reporting no change in quality of life (F(2, 80) = 5.866, P < 0.01). CONCLUSIONS: The COQOL is the first otology-specific PROM. Initial studies demonstrate excellent reliability and encouraging preliminary criterion validity: further studies will allow a deeper validation of the instrument.

Outcome of translabyrinthine surgery for vestibular schwannoma in neurofibromatosis type 2.

OBJECTIVES: To analyse the long-term outcome of translabyrinthine surgery for vestibular schwannoma (VS) in neurofibromatosis type 2 (NF2). RESEARCH TYPE: Retrospective cohort study. SETTING: Two tertiary referral NF2 units. PATIENTS: One hundred and forty eight translabyrinthine operations for patients with VS were performed. Preoperative stereotactic radiotherapy had been performed on 12(9.4%) patients. RESULTS: Mean tumour size was 3.1 cm. Total tumour excision was achieved in 66% of cases, capsular remnants were left in 24% of cases, and subtotal excision was achieved in 5% and partial removal was achieved in 5%. The radiological residual/recurrence rate was 13.9%. The perioperative mortality was 1.6%. At 2 years postoperatively, facial function was expressed in terms of House-Brackmann score (HB): HB 1 in 53.4%, HB 1/2 in 61.3%, HB 1-3 in 83.2% and HB 4-6 in 16.8%. All nine patients who underwent surgery following failed stereotactic radiotherapy had HB 3 function or better. Among 9.5% of the cases, 14 facial nerves were lost during surgery and repaired using direct anastomosis or grafting. There was no tinnitus present preoperatively in 27% of the cases, and 22% of patients developed tinnitus postoperatively. In patients with preoperative tinnitus, 61% remained the same, 17% got it resolved and only in 21% it worsened. The preoperative hydrocephalus rate was 26%, and among 15% of the cases five ventriculo-peritoneal (VP) shunts were performed. The cerebrospinal fluid leak rate was 2.5%. Fifty-six patients underwent auditory brainstem implantation (ABI) and two patients had cochlear implant (CI) sleepers inserted. CONCLUSIONS: The management of patients with NF2 presents the clinician with a formidable challenge with many patients still presenting themselves late with the neurological compromise and a large tumour load. There is still an argument for the management by observation until the neurological compromise dictates interventional treatment particularly with the option of hearing rehabilitation with ABI or CI. The translabyrinthine approach provides a very satisfactory means of reducing the overall tumour volume.

Cochlear implant failures: lessons learned from a UK centre.

OBJECTIVES: To review our experience of cochlear implant failure and subsequent revision surgery, and to illustrate the experience we have gained by presenting a series of lessons learned. METHODS: A combined retrospective and prospective study of revision surgery in a UK regional cochlear implant centre. RESULTS: Of the 746 cochlear implantations undertaken, 33 (4.7 per cent of adults and 4.1 per cent of children) had a registered failure requiring re-implantation. The mean time to device failure was 60 months in adults and 35 months in children. Causes of cochlear implant failure were medical (n = 11), electrode displacement (n = 2), 'hard device failure' (n = 15) and 'soft device failure' (n = 5). Chronic suppurative otitis media and post-auricular mastoid abscess were the commonest causes of medical failure. There was one case of electrode array displacement as a direct result of skin flap revision surgery. In 80 per cent of cases, audiological performances were stable or improved following re-implantation. CONCLUSION: As the number of cochlear implants increase and patients outlive the lifespan of their devices, we will face a growing number of revision procedures. Audiologists and otologists should be competent in diagnosing and managing device failure and medical complications requiring cochlear re-implantation.

Non echo planar, diffusion-weighted magnetic resonance imaging (periodically rotated overlapping parallel lines with enhanced reconstruction sequence) compared with echo planar imaging for the detection of middle-ear cholesteatoma.

OBJECTIVES: We evaluated use of the periodically rotated overlapping parallel lines with enhanced reconstruction diffusion-weighted imaging sequence, compared with conventional echo planar magnetic resonance imaging, in the detection of middle-ear cholesteatoma. MATERIAL AND METHODS: Sixteen patients awaiting second-stage combined approach tympanoplasty and three patients awaiting first-stage combined approach tympanoplasty underwent magnetic resonance imaging with both (1) the periodically rotated overlapping parallel lines with enhanced reconstruction sequence (i.e. non echo planar imaging) and (2) the array spatial sensitivity encoding technique sequence (i.e. echo planar imaging). Two neuroradiologists independently evaluated the images produced by both sequences. Radiology findings were correlated with surgical findings. RESULTS AND ANALYSIS: Seven cholesteatomas were found at surgery. Neither of the assessed imaging sequences were able to detect cholesteatoma of less than 4 mm. Rates for sensitivity, specificity, and positive and negative predictive values are presented. CONCLUSION: Decisions on whether or not to operate for cholesteatoma cannot be made based on the two imaging sequences assessed, as evaluated in this study. Other contributing factors are discussed, such as the radiological learning curve and technical limitations of the magnetic resonance imaging equipment.

Human auditory nerve compound action potentials and long latency responses.

CONCLUSION: The compound action potential (CAP) is followed by a long latency response (LLR), attributable to the post-auricular musculature. The LLR to one pulse may overlap with the CAP to a subsequent one, contributing to the clinically observed reduction in CAP at high pulse rates. OBJECTIVES: To measure refractory and other influences on CAPs in humans and guinea pigs. MATERIALS AND METHODS: CAPs were obtained from humans using trans-tympanic and extra-tympanic electrocochleography and from anaesthetized guinea pigs. Stimuli were single pulses presented at a slow rate, pairs of pulses, and 100 ms pulse trains where the inter-pulse interval alternated between 4 and 6 ms. RESULTS: For single pulses, the CAP shape was similar across species. For pairs of pulses, the CAP to the second pulse was smaller than that to the first, and decreased with increasing inter-pulse interval in a way that was similar across species. For pulse trains, CAPs were observed in response to each pulse in the train for the guinea pigs, but not for humans. For both filtered and unfiltered single pulses, there was a large LLR in humans, but not in guinea pigs, with peaks at latencies of 10-12 and 20-25 ms. Posture affected the LLR in a way consistent with the post-auricular response.

Superior semicircular canal dehiscence with a large tegmental defect.

We report on the presentation and clinical manifestations of superior semicircular canal dehiscence in association with a large defect of the tegmen tympani in a 41-year-old woman with no previous history of trauma. Based on this case we recommend that clinicians consider the possibility of superior semicircular canal dehiscence in patients presenting with symptoms associated with tegmen defects.

Two stage management of petrositis with associated mycotic aneurysm of the intrapetrous carotid artery.

Mycotic aneurysm of the petrous temporal bone is extremely rare, with only 12 cases previously reported. We review the literature to date and present a case of petrositis complicated by a mycotic aneurysm of the internal carotid artery, which was managed by endovascular balloon occlusion and subsequent total petrosectomy. We can find no other case in the literature where balloon occlusion has been used to treat a mycotic aneurysm of the intrapetrous carotid artery prior to total petrosectomy to treat petrositis.

Novel ATP6V1B1 and ATP6V0A4 mutations in autosomal recessive distal renal tubular acidosis with new evidence for hearing loss.

Autosomal recessive distal renal tubular acidosis (rdRTA) is characterised by severe hyperchloraemic metabolic acidosis in childhood, hypokalaemia, decreased urinary calcium solubility, and impaired bone physiology and growth. Two types of rdRTA have been differentiated by the presence or absence of sensorineural hearing loss, but appear otherwise clinically similar. Recently, we identified mutations in genes encoding two different subunits of the renal alpha-intercalated cell's apical H(+)-ATPase that cause rdRTA. Defects in the B1 subunit gene ATP6V1B1, and the a4 subunit gene ATP6V0A4, cause rdRTA with deafness and with preserved hearing, respectively. We have investigated 26 new rdRTA kindreds, of which 23 are consanguineous. Linkage analysis of seven novel SNPs and five polymorphic markers in, and tightly linked to, ATP6V1B1 and ATP6V0A4 suggested that four families do not link to either locus, providing strong evidence for additional genetic heterogeneity. In ATP6V1B1, one novel and five previously reported mutations were found in 10 kindreds. In 12 ATP6V0A4 kindreds, seven of 10 mutations were novel. A further nine novel ATP6V0A4 mutations were found in "sporadic" cases. The previously reported association between ATP6V1B1 defects and severe hearing loss in childhood was maintained. However, several patients with ATP6V0A4 mutations have developed hearing loss, usually in young adulthood. We show here that ATP6V0A4 is expressed within the human inner ear. These findings provide further evidence for genetic heterogeneity in rdRTA, extend the spectrum of disease causing mutations in ATP6V1B1 and ATP6V0A4, and show ATP6V0A4 expression within the cochlea for the first time.

'Hairy polyp' of the pharynx in association with an ipsilateral branchial sinus: evidence that the 'hairy polyp' is a second branchial arch malformation.

The pharyngeal 'hairy polyp', or 'dermoid' has caused considerable debate as to its origin since the original classification proposed by Arnold in 1870. This classification implies that the hairy polyp is either a teratoma or sequestration dermoid cyst. Many papers contest this view, in favour of a developmental malformation. We describe the first case of a hairy polyp in association with an ipsilateral branchial sinus as further supportive evidence of a developmental malformation of the second branchial arch.

Facial nerve action potentials: a study to assess waveform reliability.

OBJECTIVE: To assess the reliability of the orthodromic facial nerve action potential (FNAP), recorded from the intratemporal portion of the facial nerve on stimulation within the cerebellopontine angle. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENTS: Ten consecutive patients undergoing translabyrinthine resection of vestibular schwannoma. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURES: Ten consecutive FNAPs were recorded on stimulation of the facial nerve within the cerebellopontine angle. The FNAP recording probe was placed directly on the nerve surface after the fallopian canal was opened at the second genu. Ten consecutive compound muscle action potentials (CMAPs) were recorded simultaneously from surface electrodes overlying the facial musculature, by use of a standardized electrode placement technique. The stimulating and recording equipment were removed (excluding CMAP surface electrodes) and reapplied, and FNAP and CMAP data were recorded for a second time (test/retest). Peak-to-peak amplitudes of all waveforms were calculated. RESULTS: The average FNAP peak-to-peak amplitude for all patients was larger than the CMAP peak-to-peak amplitude (2.60 mV and 1.07 mV, respectively). Random effects analysis of variance was performed to assess the individual components of variation. This showed that CMAP was less variable than FNAP for replicate error (10 consecutive FNAPs and CMAPs) and test/retest error. However, subject variance was less for FNAP, where subject variance was by far the largest contributor to overall variation. The reliability coefficient for FNAP was 0.995 and for the CMAP was 0.982, where absolute reliability is 1.0. CONCLUSION: These data confirm that the FNAP, recorded by the technique described here, is a reliable waveform when compared with the CMAP and is a valid method for assessing facial nerve function.

Assessment of real-time clinical facial function during vestibular schwannoma resection.

OBJECTIVES: To describe a technique for quantifying residual facial function after vestibular schwannoma surgery. The intraoperative electrophysiological results are correlated with immediate postoperative clinical facial function to assess technique validity. STUDY DESIGN: Prospective blinded study. METHODS: Thirty-two patients undergoing translabyrinthine resection of vestibular schwannoma were included. Compound muscle action potential (CMAP) amplitude was calculated to supramaximal stimulation of the facial nerve, proximal to vestibular schwannoma compression, at the brain stem. The procedure was repeated after tumor removal. Comparison of the two CMAP amplitudes enabled estimation of change in facial function during surgery. The data were correlated with intracranial tumor diameter and immediate postoperative clinical facial function. RESULTS: CMAP amplitude recorded after tumor resection correlated with immediate postoperative clinical facial function (0.879, P < .01). Correlation was improved when residual facial function was calculated (0.944, P < .01). In contrast, preoperative tumor size had relatively poor correlation with immediate postoperative facial function (0.688, P < .01). CONCLUSIONS: Comparison of electrophysiological data before and after tumor removal reduces intersubject variability resulting from intersubject variation in facial muscle morphology. Residual facial function closely correlates with immediate postoperative clinical facial function, assessed using the House-Brackmann grading system. From the data presented, the technique can inform the surgeon of current clinical facial function at any point during the dissection process.

Metastatic renal cell carcinoma to the nose and paranasal sinuses.

BACKGROUND: Renal cell carcinoma represents 3% of all malignant tumors. Metastatic deposits of renal cell carcinoma to the head and neck region are infrequent. The objective of this work is to analyze the clinical presentation, radiologic features, surgical and radiotherapy treatment, and outcome of metastatic renal cell carcinoma to the nose and sinuses. METHODS: Retrospective review of 6 patients diagnosed with renal cell carcinoma who had nasal metastasis develop and were seen at the Christie Hospital in Manchester over the past 8 years. RESULTS: Six patients with renal cell carcinoma were seen with recurrent epistaxis, nasal obstruction, and unpleasant nasal crusting. Three patients had orbital involvement. Examination under general anaesthesia and biopsy was performed in all 6 cases. Histologic studies confirmed metastases of renal cell carcinoma in all 6 patients. All patients underwent local external beam radiotherapy. The most common dose used was 35 Gy in 8 daily fractions. All patients had symptomatic control of local nasal disease with a minimum follow-up of 2 years in 4 patients. Two patients died within 6 months of the radiotherapy treatment as a result of their primary tumor. CONCLUSIONS: Metastatic renal cell carcinoma to the nose and paranasal sinuses is rare but has unpleasant symptoms. Local symptomatic control with radiotherapy is excellent.

Carcinoma of the hypopharynx and cervical esophagus in young adults.

Squamous cell carcinoma of the hypopharynx and cervical esophagus usually presents in the late-middle-aged and elderly. When diagnosed in young adults, the disease process is often thought to be more aggressive and have a worse long-term outcome. Four hundred ninety patients presented to the Christie Hospital and Manchester Royal Infirmary between 1981 and 1990 with squamous cell carcinoma of the hypopharynx and cervical esophagus. Of this group, 24 patients (5%) received their diagnosis before the age of 45. A comparison is made with a control group of 156 (32%) patients who presented between the ages of 60 and 69 years. Analysis of tumor and nodal staging at presentation demonstrates no statistically significant difference between the 2 age groups. There is a higher incidence of a combination of smoking and alcohol abuse in the older age group, but it is of no statistical significance. There is no difference in 5-year survival results between the 2 groups. We conclude that patients with squamous cell carcinoma of the hypopharynx and cervical esophagus who receive their diagnosis under the age of 45 show no difference in tumor stage or long-term outcome when compared with a control group encompassing the mean age of presentation.

Facial nerve injury caused by vestibular Schwannoma compression: severity and adaptation to maintain normal clinical facial function.

OBJECTIVES: To assess facial nerve injury caused by vestibular Schwannoma compression and the adaptive ability of the nerve/muscle complex to maintain normal clinical facial function. STUDY DESIGN: Prospective study. SETTING: Tertiary referral centre. PATIENTS: Thirty-four patients undergoing translabyrinthine resection of vestibular schwannoma. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURES: Facial nerve action potential (FNAP) amplitude recorded at the second genu enables direct assessment of motoneuron function. Comparison of FNAP amplitudes to stimulation proximal and distal to tumor compression allows calculation of motoneuron conduction block across the site of tumor compression. Recordings performed before tumor dissection from the facial nerve reflects nerve injury caused by vestibular Schwannoma compression alone. Comparison of compound muscle action potential (CMAP) amplitudes to stimulation proximal and distal to tumor compression measures motoneuron conduction block and compensatory collateral sprouting at the nerve/muscle interface. Comparison of FNAP and CMAP data demonstrates the extent of collateral sprouting, which helps maintain normal clinical facial function. RESULTS: Normal clinical facial function is maintained when only 10% of functioning motoneurons are active. The facial nerve is highly susceptible to tumor compression with significant motoneuron injury occurring with all sizes of tumors. Motoneuron injury correlates with tumor size but with exceptions. Collateral sprouting maintains muscle function despite severe motoneuron injury. There is a significant decrease in muscle function when >80% of functioning motoneurons are in conduction block. CONCLUSIONS: The facial nerve is highly susceptible to vestibular Schwannoma compression. Collateral sprouting of active functioning motoneurons reinnervate denervated muscle fibers so maintaining muscle function and therefore clinical facial function.

The large vestibular aqueduct syndrome: the role of cochlear implantation in its management.

In recent years the large vestibular aqueduct syndrome has become an increasingly recognized cause of a progressive sensorineural hearing loss. Cochlear implantation, although not correcting the bony abnormality, does offer an avenue for rehabilitation for affected individuals and initial results are encouraging. Of 15 patients identified with the large vestibular aqueduct syndrome we have implanted seven (five adults and two children). All patients underwent an uneventful electrode insertion with uncomplicated postoperative periods. Three of our adult patients are showing very good initial results with BKB speech recognition scores of 100%, 74% and 66% with the implant alone. It is still early days with the children, but initial results are encouraging. A full review of the large vestibular aqueduct syndrome is given along with our results.

Imaging of temporal bone brain hernias: atypical appearances on magnetic resonance imaging.

Brain tissue with or without its dural covering may protrude into the temporal bone through a defect in the tegmen tympani. Infection or granulation tissue, with or without cholesteatoma may make diagnosis difficult. While computed tomography (CT) may demonstrate a bony defect, it is difficult to distinguish between herniated brain, cholesteatoma or granulation tissue. Magnetic resonance imaging (MRI) clearly demonstrates healthy herniated brain tissue, but ischaemic or necrotic elements may mimic other lesions. We present a series of five patients with brain hernia, highlighting the spectrum of preoperative radiological appearances, and some of the difficulties encountered in interpreting these images. MRI demonstrated herniation of healthy brain in two patients but in three, showed irregular soft tissue with signal characteristics consistent with cholesteatoma. Surgery in these three patients demonstrated necrotic brain with coexisting cholesteatoma in two. Brain hernias are often necrotic and may have morphological and signal characteristics similar to cholesteatoma with which they may coexist.

Petrosal cholesteatoma: management considerations for minimizing morbidity.

OBJECTIVE: This study aimed to analyze the surgical treatment of patients presenting with petrosal cholesteatoma. STUDY DESIGN: The study design was a retrospective case review. SETTING: The study was conducted at a tertiary referral center. PATIENTS: Patients were referred to the University Department of Otolaryngology, Manchester Royal Infirmary, with surgically confirmed petrosal cholesteatoma. INTERVENTION: Interventions were diagnostic and therapeutic. MAIN OUTCOME MEASURES: Postoperative facial function, hearing loss, recurrent cholesteatoma, and petrous cavity morbidity in relation to preoperative signs, intraoperative findings, and surgical techniques were measured. RESULTS: Twenty-five patients were treated between 1979 and 1997. Complete preoperative facial paralysis was always associated with bony erosion around the geniculate ganglion, and facial nerve ischemia was thought to be an important factor. Resection of the ischemic nerve segment and end-to-end anastomosis over the posterior fossa dura allowed full removal of cholesteatoma matrix and reinnervation along a healthy and vascularized nerve. In five of the seven cases with recurrent disease, cholesteatoma was localized to the intrapetrous carotid or geniculate ganglion. The introduction of endoscopic techniques has allowed assessment of the full extent of disease and removal of cholesteatoma matrix missed using conventional microscopic visualization. Bipolar diathermy to areas of cholesteatoma, densely adherent to dura, is effective in destroying squamous epithelium. Patients with cavity obliteration and blind sac closure had minimal postoperative morbidity, and current magnetic resonance imaging techniques are effective in diagnosing and delineating recurrence. CONCLUSIONS: This study illustrates a gradual change in the treatment of patients with petrosal cholesteatoma. By defining the main areas of patient morbidity and using both recent technical advances and alternative surgical techniques, further improvement in patient outcome is envisaged.

Auditory rehabilitation in neurofibromatosis type 2: a case for cochlear implantation.

Cochlear implantation has a limited but definite role in the rehabilitation of certain neurofibromatosis type 2 (NF2) patients. The presence of a dead ear either before, or after, tumour removal does not necessarily imply loss of function in the eighth nerve; in some instances the hearing loss will be cochlear. Promontory or round window electrical stimulation may help to identify those individuals with surviving eighth nerve function. In such patients multichannel cochlear implantation promises a better level of audition than the auditory brain stem implant. This paper highlights such a case and the management problems are discussed.