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.

Providing an extended use of an otological-specific outcome instrument to derive cost-effectiveness estimates of treatment.

OBJECTIVES: Although quality-adjusted life years (QALYs) are increasingly being used by decision-makers to make comparisons of cost-effectiveness, there are no otological-specific outcome measures that fit within this QALY framework. This study had two main objectives. The first was to provide a means to derive QALYs from a condition-specific otological instrument (Cambridge Otology Quality of Life, COQOL), and the second was to assess the convergent validity, or degree of correlation, between the COQOL and SF-6D, an established QALY instrument. DESIGN: Longitudinal cohort study designed to assess the convergent validity between SF-6D and COQOL and to generate a mapping function to enable SF-6D values to be predicted from the COQOL responses. SETTING: Cambridge University Hospital, UK. PARTICIPANTS: A total of 207 patients attending a routine outpatient general otology clinic. MAIN OUTCOME MEASURES: SF-6D and the COQOL instrument completed at baseline and again 3 months later. RESULTS: Convergent validity was demonstrated with mean SF-6D values decreasing linearly with increasing severity on the COQOL instrument. Overall, the correlation between the COQOL scores and the SF-6D values was moderate and statistically significant (r = 0.490, P = <0.001). A simple mapping model based on an ordinary least squares (OLS) regression function predicted SF-6D values from the COQOL data with a reasonable degree of accuracy. Further validation using the follow-up 3-month data confirmed the prediction power of this mapping model. CONCLUSIONS: This study provides a method for estimating QALYs from condition-specific COQOL data and provides the opportunity for the cost-effectiveness of otological treatment to be measured and placed within the national QALY framework.

Conservative management, surgery and radiosurgery for treatment of vestibular schwannomas: a model-based approach to cost-effectiveness.

OBJECTIVE: To undertake a cost-effectiveness analysis comparing conservative management, surgery and radiosurgery for treating small-to-medium (1-20 mm)-sized vestibular schwannomas. DESIGN: Model-based economic evaluation using individual-level data from a Birmingham-based longitudinal patient database and from published sources. Both a decision tree and state-transition (Markov) model were developed, from an National Health Service (NHS) perspective. Sensitivity analyses were also carried out. SETTING: Secondary care treatment for patients with small-to-medium-sized vestibular schwannomas. PARTICIPANTS: Three hypothetical cohorts of adult patients receiving conservative management, radiosurgery or surgery treatment, aged 58 years as starting age within model. MAIN OUTCOME MEASURES: Cost-effectiveness based on cost per quality-adjusted life year (QALY). RESULTS: Conservative management is the preferred strategy for the treatment of small-to-medium-sized vestibular schwannomas. Conservative management is both cheaper (-£ 722 and -£ 2764) and more effective (0.136 and 0.554 quality-adjusted life years) than both radiosurgery and surgery, respectively. A conservative strategy can therefore be considered as highly cost-effective. This result is sensitive to the assumed quality-of-life parameters in the model. Sensitivity analysis suggests that the probability of a conservative strategy being the most cost-effective approach compared with surgery and radiosurgery at a willingness to pay of £ 20 000/quality-adjusted life year gained is 80% and 55%, respectively. CONCLUSIONS: A conservative approach is the preferred strategy for treatment of small-to-medium vestibular schwannomas. This result is sensitive to quality-of-life values used in the analysis. More research is required to assess the impact of treatment upon patients' health-related quality of life over time.

Open and closed cavity mastoid operations: comparing early hearing results.

The aim of the study is to compare audiological results in patients treated surgically with either an open or closed cavity mastoidectomy. A prospective review of a single surgeon's case series based at two district general hospitals. All patients with 1-year post-operative hearing results following an open or closed cavity mastoid surgery were included. Outcome measured included the preoperative and postoperative air-bone gap (ABG). Belfast rule of thumb was used to assess the benefit from middle ear surgery. A total of 128 patients were identified from the database with 1 year post-operative hearing results. Sixty-three patients had undergone an open cavity mastoidectomy and 65 had a closed cavity mastoidectomy. The mean post-operative ABG in patients with open cavity mastoidectomy was 24 dB and for closed cavity 21 dB (p = 0.12). There was no statistical difference between open and closed cavity mastoidectomy with or without ossiculoplasty and if the underlying pathology was squamous or mucosal disease. However, patient with post-operative ABG below or equal to 20 dB was 41% for closed cavity compared to 21% for open cavity. Using the Belfast rule of thumb, 79% of the patients in both groups had successful hearing results post surgery. The 1-year post-operative audiological results between open and closed cavity masoidectomy showed no statistical difference. The pathology affecting the middle ear also had no influence on the hearing results between the two groups. However, there was a higher percentage of patients with closed cavity procedures who had a post-operative ABG that was below or equal to 20 dB.

Facial nerve outcomes in functional vestibular schwannoma surgery: less than total tumour excision significantly improves results.

OBJECTIVES: To determine the implications of a functional approach to vestibular schwannoma surgery, with facial nerve function prioritised higher than total tumour excision. STUDY DESIGN: A case-control study in a tertiary referral neurotology clinic. PATIENTS: A 'functional' surgical group treated after April 2007 (n = 44, mean cerebellopontine angle dimension 27 mm), and an 'excisional' surgical group matched for tumour size, treated from 1997 to April 2007 (n = 115). INTERVENTION: Change to more functional surgical approach. PRIMARY OUTCOME: facial nerve status. Secondary outcome: tumour recurrence in less-than-total tumour excision. RESULTS: Facial nerve preservation: 77 per cent House-Brackmann grade I-II in functional group at 12 months, versus 57 per cent grade I-II in excisional group (p = 0.027). Tumour recurrence: 1 per cent in total excision group, 2 per cent in near-total group and 40 per cent in sub-total group. CONCLUSION: A functional approach to vestibular schwannoma surgery improves facial nerve preservation outcomes and reduces the requirement for facial nerve rehabilitative interventions. Tumour recurrence rates are low in near-totally excised lesions but significant if only sub-total excision is achieved.

Chronic suppurative otitis media in Nepal: ethnicity does not determine whether disease is associated with cholesteatoma or not.

OBJECTIVES: To determine the frequency of cholesteatoma in a population of patients with chronic suppurative otitis media, and to determine whether this frequency is affected by ethnicity. PATIENTS: The study included 6005 patients with chronic suppurative otitis media seen during the course of 30 charitable surgical 'ear camps' in Nepal. MAIN OUTCOME MEASURES: Proportion of patients with each subtype of disease, and their ethnicity. A secondary outcome measure was concordance of surname with Nepalese ethnic affiliation. RESULTS: A total of 762 patients were grouped as being of Tibeto-Mongolian origin, and 4875 as Indo-Caucasian. The rate of chronic suppurative otitis media with cholesteatoma, expressed as a proportion of the rate of all chronic suppurative otitis media subtypes, was 17.8 per cent in Tibeto-Mongolian patients and 18.6 per cent in Indo-Caucasian patients (p > 0.05). The effect of other risk factors (i.e. age, gender and geographical district) on disease distribution was also non-significant. Analysis of secondary outcome measures indicated that patients' surnames were a reliable predictor of ethnicity in this Nepalese population. CONCLUSION: There is almost complete concordance in proportions of patients with significant genetic, cultural, and even geographical heterogeneity, suggesting that, in Nepal, the aetiology of cholesteatoma owes little to these factors.

The bone-anchored hearing aid in the rehabilitation of single-sided deafness: experience with 58 patients.

OBJECTIVES: To assess the efficacy of the bone-anchored hearing aid (BAHA) in the rehabilitation of single-sided deafness (SSD). STUDY DESIGN: Retrospective case-control series review. SETTING: Tertiary referral unit. PATIENTS: Fifty-eight consecutive patients that had a bone-anchored hearing aid for single-sided deafness completed outcome questionnaires, building upon earlier audiological assessment of 19 patients. Single-sided deafness controls (n = 49) were mainly acoustic neuroma patients. MAIN OUTCOME MEASUREMENTS: speech discrimination testing in directional noise, speech and spatial qualities of hearing questionnaire and the Glasgow Benefit Inventory (GBI). RESULTS: The mean follow-up time was 28.4 months. Five (13%) of the bone-anchored hearing aid patients were non-users because of lack of benefit. The audiometric testing confirmed that when noise was on the bone-anchored hearing aid side speech perception was reduced but benefited when noise was on the side of the hearing ear. There was no difference between the Speech and Spatial Qualities of Hearing Scores in bone-anchored hearing aid users and controls. In particular there was no difference in the spatial subscores. In the bone-anchored hearing aid users the median Glasgow Benefit Inventory score was 11. If the non-users are included then 13 (22%) patients had no or detrimental (negative) Benefit scores. No or negative benefit scores were more frequent in those deaf for <10 years. In open-field questions, patients felt the bone-anchored hearing aid was most useful in small groups or in 'one-to-one' conversation. CONCLUSIONS: Bone-anchored hearing aid rehabilitation for single-sided deafness is less successful than for other indications, reflected here by relatively low median Glasgow Benefit Inventory scores. There was also no significant difference between controls and bone-anchored hearing aid users in the Speech and Spatial Qualities of Hearing Questionnaire. Patients with a longer duration of deafness report greater subjective benefit than those more recently deafened, perhaps due to differing expectations.

A case of paraganglioma of the hypoglossal nerve.

INTRODUCTION: Paragangliomas are rare tumours arising from the paraganglia of the autonomic nervous system. CASE REPORT: We present a case of a paraganglioma arising from the hypoglossal nerve and producing an unusual clinical picture at presentation. DISCUSSION: We supply radiological evidence of a paraganglioma originating from the hypoglossal nerve, and thus extend the evidence base for this rare site of origin. Our patient presented as an emergency with long tract neurological symptoms and progressive brainstem involvement. This presentation is not characteristic of paragangliomas in general, which usually have an indolent growth pattern and often demonstrate benign symptoms for a number of years prior to diagnosis. The location of a hypoglossal paraganglioma differs significantly from more common paragangliomas described in the neck and skull base, and this should inform the surgical approach undertaken.

Results of primary ossiculoplasty in ears with an intact stapes superstructure and malleus handle: inflammation in the middle ear at the time of surgery does not affect hearing outcomes.

OBJECTIVE: To determine whether any factors are predictive of success or failure in primary ossiculoplasty employing autologous bone in Austin-Kartush Group A (stapes and malleus handle present) ears. STUDY DESIGN: Retrospective review of a single-surgeon case series. SETTING: Two District General Hospitals in the United Kingdom. PATIENTS: Of 135 patients undergoing primary repair, 116 had sufficient data for analysis (mean follow-up 33 months). MAIN OUTCOMES MEASURED: Postoperative air-bone gaps (pABG) in patients with differing qualities of middle-ear mucosa (active versus inactive), differing pathologies (mucosal versus squamous) and differing surgical procedures [canal-wall up (CWU) versus canal-wall down (CWD)]. SECONDARY OUTCOME MEASURE: 'Belfast Rule of Thumb' (operated ear hearing brought to 30 dB or better or to within 15 dB of contralateral ear). RESULTS: Postoperative ABGs were closed within 10 dB in 37%, 20 dB in 74% at latest follow-up. The only significant factors predicting hearing outcomes were the status of the canal wall (CWU = 14.1 dB, CWD = 24.7 dB: significant, P = 0.00) and whether surgery was primary or revision. Mean ABG was 14.7 for inactive middle ear mucosa, 17.2 for active middle ear mucosa (not significant, P = 0.25). Excluding CWD procedures from the analysis improved outcomes to 80% (within 20 dB) and 41% (within 10 dB) overall, and the mean for active and inactive ears to 14.2 and 14.0 dB respectively. Postoperatively, 82% of patients fulfilled the 'Belfast Rule of Thumb'. CONCLUSION: The most significant prognostic factor affecting hearing outcomes - status of the canal wall - is independent of staging. The factor most associated with staging - the status of the middle ear mucosa - is only weakly associated with a successful functional outcome.

Conservative versus primary surgical treatment of acoustic neuromas: a comparison of rates of facial nerve and hearing preservation.

OBJECTIVES: To determine whether patients with small to medium sized acoustic neuromas managed conservatively suffer reduced rates of facial nerve and hearing preservation when compared with patients undergoing primary surgery. DESIGN: An intention-to-treat comparison between patients managed conservatively at first presentation and those managed with primary surgery. SETTING: Tertiary referral neurotological centre in Birmingham, UK. PARTICIPANTS: Data were drawn from our database of 487 sporadic acoustic neuromas managed from 1997 to present day. MAIN OUTCOME MEASURES: Facial nerve status (assessed using the House-Brackmann system) was collated for all conservatively managed patients (n = 167) and compared with that expected when calculated from primarily surgically managed tumours of equivalent size (n = 121). A chi-square test was employed to test the statistical significance of any difference. Hearing preservation (maintenance of AAO-HNS Class) in patients presenting with Class A or B hearing was compared between conservatively managed patients and those with primary surgical management. RESULTS: Observed facial nerve preservation in conservatively managed patients was significantly better (P < 0.001) than expected when calculated from rates of facial nerve preservation in surgically managed patients. Hearing preservation was also significantly more successful in conservatively managed patients (Pearson chi-square: P < 0.000). CONCLUSIONS: An initial period of conservative management is a safe and reasonable management policy in all acoustic neuromas up to 2 cm in size at the cerebello-pontine angle. Given that there are no failsafe ways of deciding potential for growth in acoustic neuromas, initial conservative management of these tumours offers superior hearing and facial nerve preservation when compared with primary surgical treatment.

The genetics of paragangliomas: a review.

Familial head and neck paragangliomas account for approximately 10% of all head and neck paragangliomas. There are three known genes associated with genetic susceptibility to head and neck paragangliomas: succinate dehydrogenase complex subunit 'D', 'B' and 'C' (SDHD, SDHB and SDHC). The genes most frequently implicated: SDHD and SDHB, also predispose to phaeochromocytoma. SDHD shows a complex inheritance pattern - tumours do not develop if the mutation is inherited from the mother. SDHB mutations are associated with malignant phaeochromocytoma. Patients who present with a family history of paraganglioma or phaeochromocytoma, with multiple tumours, or early onset tumours (<50 years), should be referred for genetic investigation.

A large and uncharacteristically aggressive cholesterol granuloma of the middle ear.

Cholesterol granulomas of the middle ear tend to be benign in nature, frequently seen by otologists during cholesteatoma or chronic otitis media surgery and easily dealt with by evacuation. In contrast, cholesterol granulomas of the petrous apex appear to have a more aggressive nature and present significant pathological threats. We present a case of a large destructive cholesterol granuloma of the middle ear eroding the middle and posterior cranial fossa bone and dura to exert a mass effect upon the temporal lobe. We emphasize the destructive potential of this extremely rare middle-ear cholesterol granuloma, and draw attention to the distinction between this variant and the commonplace and benign form of the lesion more usually found at this site. We also present the current theories of the aetiology of cholesterol granuloma formation with possible explanations for the rare aggressive behaviour.