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Main Article The Journal of Laryngology & Otology (2009) , 123 , 502508 . # 2008 JLO (1984) Limited doi:10.1017/S0022215108003575 Analysis of causes for late presentation of Indian patients with vestibular schwannoma R A MBETT , V R UPA ,


  1. Main Article The Journal of Laryngology & Otology (2009) , 123 , 502–508 . # 2008 JLO (1984) Limited doi:10.1017/S0022215108003575 Analysis of causes for late presentation of Indian patients with vestibular schwannoma R A MBETT , V R UPA , V R AJSHEKHAR * Abstract Objective: To determine the causes of delay in diagnosis and treatment of Indian patients with vestibular schwannomas. Methods: In a prospective study from 2003 to 2005, 50 patients with a confirmed diagnosis of vestibular schwannoma were interviewed to determine the causes for (1) the delay between the patient noting the initial symptom and the definitive diagnosis, and (2) the reasons for delayed diagnosis. Results: In 90 per cent of patients, the initial symptom was either hearing loss (62 per cent), vertigo (24 per cent) or tinnitus (4 per cent). However, most patients had been diagnosed and had presented for surgery only after neurological symptoms had became apparent. The delay between the initial medical consultation and the final diagnosis ranged from one month to 204 months (mean + standard deviation, 32.2 + 38.9 months). After the patient had noted symptoms, the diagnosis of vestibular schwannoma was delayed due to doctor-related causes in 80 per cent of cases, and due to patient-related causes in 20 per cent. Delay following diagnosis was minimal. Conclusions: Delay in the diagnosis of vestibular schwannoma in Indian patients is due to both doctor- and patient-related factors. Key words: Sensorineural Hearing Loss; Vestibular Schwannoma; Diagnosis; India Introduction such as Denmark with well organised referral Vestibular schwannoma is a benign brain tumour, systems, an increasing number of patients with and its site of origin and propensity to produce tumours . 40 mm in size were being seen. They symptoms potentially make early detection and attributed this to the fact that unilateral, progressive complete resection a definite possibility. In actual hearing loss was still a neglected symptom, both by patients and doctors. Moffat et al. 5 reported in 2004 fact, however, many patients present late in the course of the disease with increased pre- and post- that in spite of the easy availability of MRI scanning operative morbidity. 1 Unilateral or asymmetrical facilities and a good referral system in their part of audiovestibular symptoms were the earliest com- the UK, they continued to see patients with giant ves- plaints in over 75–90 per cent of patients in tibular schwannomas, with increasing frequency. some series. 1 – 3 The earliest opportunity for diag- They felt that many patients with large tumours had nosis, therefore, lies with the ENT surgeon. probably been asymptomatic in the early stages. In Awareness of the need for screening for vestibular the Indian context, where resources and factual infor- schwannoma in such patients should prompt mation on health and disease are limited in many appropriate referral for audiological and radiologi- parts of the country, this problem is even more pro- cal tests. nounced. Consequently, the average size of the The advent of gadolinium magnetic resonance tumour seen in many case series from different imaging (MRI) scanning of the brain has enabled parts of the country, including our own institution, the diagnosis of tumours as small as 2 mm. 4 Yet, is . 35 mm. patients continue to present late with multiple In order to determine the medical and social cranial nerve palsies, cerebellar signs and features factors responsible for late presentation of Indian of raised intracranial pressure. In 1990, Thomsen patients with vestibular schwannoma, a prospective and Tos 3 lamented the fact that, despite a nation- study of 50 patients with radiologically and histologi- wide, intensive campaign to diagnose vestibular cally proven vestibular schwannoma was undertaken schwannoma at an early stage, in a small country at our institution. From the Departments of ENT and *Neurological Sciences, Christian Medical College, Vellore, India. Accepted for publication: 30 June 2008. First published online 23 September 2008. 502 Downloaded from https://www.cambridge.org/core. IP address: 192.151.151.66, on 23 Oct 2020 at 13:37:23, subject to the Cambridge Core terms of use, available at https://www.cambridge.org/core/terms. https://doi.org/10.1017/S0022215108003575

  2. 503 LATE PRESENTATION OF INDIAN PATIENTS WITH VESTIBULAR SCHWANNOMA Materials and methods TABLE I PRESENTING SYMPTOMS IN PATIENTS � WITH VESTIBULAR The study was conducted prospectively at Christian SCHWANNOMA Medical College, Vellore, India, between 2003 and 2005. We included all patients who were admitted Presenting symptom n (%) to the neurosurgical ward or who presented to the Hearing loss 31 (62) ENT out-patient clinic for evaluation and treatment Vertigo 12 (24) of a cerebellopontine angle mass diagnosed by MRI Tinnitus 2 (4) or computed tomography (CT) scanning of the brain. Headache 5 (10) Any patient who was subsequently found not to have Total n ¼ 50. a histological diagnosis of vestibular schwannoma was excluded from the study. Patients were interviewed with regard to the degree of hearing loss. Hearing loss was generally nature and duration of their presenting symptom, found to be severe (38 per cent) or profound (44 delay between first symptom and first medical per cent) in those complaining of this symptom. contact, and the type of doctor consulted initially The sole patient with normal hearing had vertigo and subsequently until definitive diagnosis and treat- alone as his presenting symptom. ment. Other data recorded comprised: duration of delay between initial symptom onset and definitive Frequency of telephone use vs presence of hearing diagnosis; reason for delayed diagnosis; type of loss. We hypothesised that frequent telephone use misdiagnosis; and duration of delay between defini- would lead to increased awareness of hearing loss. tive diagnosis and treatment. Specific note was Twenty-four patients had used the telephone regu- made of whether the patient used a telephone regu- larly, of whom 17 (68 per cent) complained of larly. We recorded the type and results of investi- hearing loss. On audiometric evaluation, most gations ordered prior to definitive diagnosis, patients who used the telephone regularly (i.e. 24 namely: CT or MRI scan of the brain, pure tone patients). (83.3 per cent) had hearing loss in the audiometry (PTA), and brainstem auditory evoked affected ear, which was either severe (50 per cent) response test audiometry. We also recorded the size or profound (33.3 per cent). Of the 26 patients who of the tumour on contrast-enhanced MRI or CT did not use the telephone regularly, 14 (54 per scan of the brain in the immediate pre-operative cent) complained of hearing loss. Although a slightly period, measured in millimetres and categorised higher proportion of those using the telephone com- as , 20 mm, 20–40 mm and . 40 mm. plained of hearing loss, this difference was not stat- All 50 patients included in the study underwent istically significant (chi-square test; p ¼ 0.90). complete otoneurological examination and audio- metric investigation. The pure tone average was cal- culated as the average of audiometric thresholds at Vertigo. Vertigo was the second most common 500 Hz and at 1, 2 and 4 kHz, and was categorised presenting symptom, with 12 (24 per cent) of the as normal ( , 20 dB), mild (25–40 dB), moderate to 50 patients seeking help for this. Seven of these severe (45–90 dB) and profound ( . 90 dB) hearing 12 patients had seen a general practitioner and loss. Brainstem auditory evoked response testing five had consulted a specialist primarily for this was performed in those who had sufficient residual symptom. Specialists seen included a neurologist, hearing. a cardiologist, a physician, a gynaecologist and a general surgeon. None of those presenting with vertigo went directly to an ENT specialist. Interest- Statistical analysis ingly, only five patients with vertigo as a presenting The mean, median, mode and standard deviations symptom were secondarily referred to an ENT were calculated for all the parameters studied. The specialist. However, the ENT surgeon undertook Student t -test was performed to determine the signifi- correct investigation and arrived at a diagnosis in cance of regular telephone use on awareness of only one of these patients. hearing loss. Tinnitus. Two (4 per cent) of the 50 patients pre- sented with an initial symptom of tinnitus. One of Results these two had giddiness along with tinnitus and Patient demographics hence ignored the latter symptom. The other The study included 50 patients, 32 males (mean 41.3 patient had associated hearing loss, for which he con- years, range 15–79 years) and 18 females (mean 42.6 sulted an ENT specialist, demonstrating that unilat- years, range 22–76 years). Forty-six per cent of eral tinnitus is often neglected as a symptom. patients were from rural areas. Headache and other non-otological symptoms. Five Initial symptoms (10 per cent) of the 50 patients presented initially Hearing loss. Hearing loss was the initial symptom in with headache; these patients were seen initially by 31 (62 per cent) patients, and was the only presenting either a general practitioner or a neurologist and symptom in 14 (28 per cent) (see Table I). Audiolo- were then referred to a neurologist or neurosurgeon gical testing showed that 49 of 50 patients had some for definitive evaluation. Several patients presented Downloaded from https://www.cambridge.org/core. IP address: 192.151.151.66, on 23 Oct 2020 at 13:37:23, subject to the Cambridge Core terms of use, available at https://www.cambridge.org/core/terms. https://doi.org/10.1017/S0022215108003575

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