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Practice Rational Testing

Investigating sudden hearing loss in adults

BMJ 2018; 363 doi: (Published 12 November 2018) Cite this as: BMJ 2018;363:k4347

Re: Investigating sudden hearing loss in adults

Dear Mr Fishman and Dr Cullen,

Thank you for writing this exhaustive article on sudden hearing loss which will be a useful guide to primary care, emergency department and walk in centre clinicians (and ENT juniors alike).
It seems especially timely given 2 publications in the last few months were independently advocating for direct to audiology referral systems, from colleagues in Brighton and Southend, who demonstrated even further streamlined patient pathways for otological emergencies.

In our practice in Merseyside, clinicians regularly tell us that tuning forks, let alone audiometry facilities, are not always available in many primary care or emergency settings.
In these circumstances, we recommend use of the previously described “hum test” (Rauch, 2008) in your armoury of techniques to differentiate between sensorineural and conductive hearing loss. . Humming heard in the patient's good ear suggests sensorineural deafness in the deaf ear, whereas humming heard in the deaf ear suggests a conductive loss in the deaf ear.

We were interested to read your inclusions of apoplexy and Cogan's syndrome among common causes of adult onset hearing loss, and enjoyed delving into the textbooks to read about these conditions.
Although qualitative descriptors of disease incidence are subjective and open to interpretation, we would like to reassure readers that these pathologies can be reassuringly consigned to ‘rare’ causes. Cogans syndrome has approximately 300 case reports ever, and an approximate prevalence of 40 patients in France (pop 67 million) (doi: 10.1016/j.autrev.2017.10.005. Epub 2017 Oct14, doi:10.1016/j.amsu.2018.04.030)
Pituitary apoplexy has an incidence of 6.2 patients per population of 100,000 amongst the GPs of Banbury (doi: 10.1111/j.1365-2265.2009.03667.x. Epub 2009 Jul 24)- yet reviews of case series do not cite hearing loss as a presenting symptom.

Given these inclusions, we were therefore a little concerned that systemic autoimmune disorders such as Granulomatosis with Polyangiitis and Eosinophilic Granulomatosis with Polyangiitis were not included as causes of sensorineural hearing loss. We accept that they are also not particularly common causes of hearing loss but hearing loss can be a first presentation and a paper last year estimated 700 new UK cases of GPA each year.

The issue that concerns us most is Box 3 - Red Flags for Referral. Urgency of this "Red Flag" referral speed is poorly defined, and could lead to a two-week rule cancer referral and we remain concerned that not all symptoms had asterisked explanations at the bottom of the box.
* Sudden Sensorineural Deafness is an emergency (not a red flag) and we echo the previous responses in recommending immediate treatment with 60mg prednisolone for one week +/- tapering thereafter to be started as soon as the diagnosis is made and ideally within 3 days of onset; rather than a "short course of oral steroids may be offered...within 14 days" as suggested in your article.
* Progressive hearing loss i.e. worsening over time, could be true of presbyacusis. We question recommending that GPs refer all such patients as a 2-week rule.
* Unilateral/asymmetric hearing loss (which could be an acoustic neuroma) should not be a red flag. An acoustic neuroma is a benign and very slowly growing tumour and does not constitute anything other than a routine referral. Our two-week wait clinic already has far too many patients with unilateral deafness in it and your article is likely to increase these numbers significantly such that we cannot see those patients who do genuinely have "red flag" symptoms for Head & Neck cancer.
* Bilateral and profound hearing loss. As we mentioned earlier, most GPs do not have audiology to be able to make this diagnosis. And we would argue that, whilst it can be very disabling to patients, it does not constitute a red flag two-week referral either.
* Hearing loss associated with other symptoms such as otorrhoea or facial palsy. This should be an immediate referral/ phone discussion to on-call rather than a two week wait.
* Hearing loss associated with head trauma. This is a potential emergency not a red flag for an outpatient clinic. The GP must send the patient to A+E for a review regarding CT scan.
* Hearing loss In an immunocompromised patient with otalgia and otorrhoea. We presume you are suggesting benign necrotising otitis externa. This should also be an immediate referral to ENT on call.

We welcome any further dialogue with yourself and our non-ENT colleagues on this issue.

Competing interests: No competing interests

09 December 2018
Thomas Hampton
Mr Chris Webb, ENT Consultant
Royal Liverpool & Broadgreen University hospitals Trust