Recurrent ear bleed with profound bilateral sensorineural hearing loss: A case of Munchausen syndrome.

Factitious disorders and Munchausen syndromes present with history and physical symptoms to all specialties, and they are often extensively evaluated. Diagnosis of Munchausen syndrome is a challenge and patients often do not receive the correct diagnosis and appropriate care especially in settings where access to mental health professionals is difficult. We present a case of recurrent bleed from the right ear, bilateral profound hearing loss and jerky movement of limbs that was extensively evaluated and followed up for 4 years until a diagnosis of Munchausen syndrome was reached. This case reports the risk of harm to the patient and wastage of healthcare resources unless physicians begin to actively evaluate for factitious disorders.

Introduction

Munchausen syndrome refers to those with severe and chronic forms of factitious disorders.[1] Munchausen syndrome is characterized by repeated fabrication of clinically convincing simulations of disease, feigning acute medical or surgical illness and giving false and fanciful information about their medical and social background. Such patients often undergo painful or risky tests and surgeries in order to obtain the sympathy and special attention given to people who are truly ill. A review of records from 2008 to 2016 reported that the incidence of factitious diseases was 3.71 per 100,000 in Germany and 3.18 in Norway with almost equal gender distribution.[2] Data on the population-level incidence of Munchausen syndrome are lacking and existing case reports are mostly based in developed countries.

While most physicians are aware of factitious disorders and Munchausen syndrome, it is often misdiagnosed for long periods of time.[2] We present a case of Munchausen syndrome in a middle-aged male who had visited multiple hospitals and was extensively evaluated for 4 years for recurrent bleeding from the right ear, hearing loss, abnormal jerky movement of limbs and poor sleep. The patient was initially evaluated and managed by the ENT team and only after 4 years did the patient have access to a psychiatrist. We discuss this case in the background of a free health care system in Bhutan, a lower-middle-income country with a population of 0.7 million and only three psychiatrists, and the need for timely access to mental health professionals in the early part of the disease course.

Case report

A 46-year-old male, who works as an instrument calibrator in a private company, presented to a district hospital 4 years ago with a history of fall at workplace. He had reported a loss of consciousness and bleeding from the right ear and was managed symptomatically. Following his discharge, he had multiple hospital visits with complaints of recurrent bleed from his right ear and loss of hearing in bilateral ears. There was no history of ear pain, pruritus, tinnitus or use of cotton buds to clean the ears. After a year, he was referred to the National Referral Hospital, Thimphu, Bhutan.

Initial otoscopy and microscopy examination showed extensive scratch marks and blood clots in the posteroinferior quadrant of the external auditory canal in the right ear (Figure 1); the rest of the physical findings were normal. His blood counts, liver and renal functions, coagulation profile were normal and was newly diagnosed with type 2 diabetes mellitus.

Figure 1.

Endoscopic photograph that showed extensive scratch mark with blood clots in posteroinferior quadrant of the external ear canal with a normal-looking tympanic membrane in the right ear.

Pure tone audiogram showed profound sensorineural hearing loss in the right (affected side) and severe sensorineural hearing loss in the left ear. He was given a hearing aid for the left ear free of cost from the hospital. He presented again with repeated ear bleed and examination under anaesthesia was done, the external ear canal skin was transected and tympanomeatal flap raised and the bony canal and middle ear were inspected and no abnormality was noted. He presented again later with right ear bleed, his high-resolution computed tomography (HRCT) of the temporal bone was a normal study. He underwent a cortical mastoidectomy to rule out any undiagnosed pathology. Despite normal findings on extensive ENT review, he continued to complain about bleeding from his right ear.

During his hospital stay, he had difficulty in sleeping and reported jerky movement of all four limbs with preserved consciousness and no tongue bite, frothing, rolling of eyeballs, or bladder or bowel incontinence. A neurology review with electroencephalogram and video telemetry ruled out seizures. Magnetic resonance imaging (MRI) brain, cerebral angiogram and a cardiology review with 2D echo showed normal studies. While in the ward, he had abnormal behaviours such as staring at objects and refusing to take medications. What drew our attention was that such attacks were more frequent whenever he was advised discharge from the hospital.

Although he was using a hearing aid, in an unusual observation, he was able to hear soft voice and when the physician talked from behind. Pure tone audiometry showed bilateral profound sensorineural hearing loss out of proportion to his ability to communicate without lip reading. A battery of tests with the otoacoustic emission, acoustic reflex, and brainstem evoked response audiometry with tone bursts suggested only high-frequency hearing loss.

He had separated from his wife 5 years ago and had financial problems educating his three children. While he had multiple hospital visits and admissions, he continued to receive a salary from his employer. Based on the clinical history, examination, extensive evaluation and follow-up over 4 years and a psychiatry review, a diagnosis of Munchausen syndrome was made. He was followed up with monthly visits at the psychiatry outpatient clinic and did not have recurrent ear problems.

Discussion

This is a case of Munchausen syndrome confirmed 4 years after the presentation and multiple hospital visits, admissions and extensive evaluation. He had initially presented to a district hospital and was subsequently referred to the National Referral Hospital. This case reflects the prolonged period of risk endured by the patient and wastage of healthcare resources. During his 4-year follow-up, he was exposed to risks of iatrogenic complications arising from unnecessary tests and treatments. The cost of all his medical visits, investigations, procedures and hearing aid were borne by the free healthcare system of the country.

Persons with factitious disorders deliberately simulate or exaggerate symptoms of an illness in themselves and can mislead healthcare workers. Factitious disorder differs from malingering in that it is not done for obvious external rewards such as financial gain, housing, or medications.[3,4] The Diagnostic and Statistical Manual of Mental Disorders (fifth edition) diagnosis requires the following: falsification of physical or psychological signs or symptoms or induction of injury or disease associated with identified deception; the individual presents as ill, impaired or injured; the deceptive behaviour is evident even in the absence of obvious external rewards; and the behaviour is not explained by another mental disorder such as delusional disorder or another psychiatric disorders.[1,3]

People with factitious disorders have an inner need to be seen as ill or injured, but not to achieve a concrete benefit, such as a financial gain.[1] They present to all medical specialties.[1,5] While it was earlier believed that a vast majority of patients with factitious disorder are female, a review of records from Germany and Norway reports a nearly equal gender distribution.(Geile, Aasly and Madea, 2020) The average age of presentation is between 30 and 40 years of age, and like other patients with factitious disorders, they tend to have either direct or peripheral knowledge of the medical field.[6] Munchausen syndrome was described initially in adults, it is reported by proxy in children.[7,8]

Patients with Munchausen syndrome commonly have a history of multiple hospitalizations, usually for acute problems.[1] Because of multiple presentations and diagnostic tests they undergo, the physical examination generally reveals multiple bodily scars.[1] Unlike patients with factitious disorders who present with what appears to be normal medical problems, patients with Munchausen syndrome present with unusual and dramatic presentations and histories that are often inconsistent with physical examination or laboratory evaluations. They offer no resistance or concerns when diagnostic procedures are offered, no matter how invasive they may be.[7,8]

While the most common falsified symptoms include abdominal pain, arthralgia, chest pain, bleeding, diarrhoea, haematuria and seizures,[1,7,8] the two main ENT regions involved are the face and ear.[9] The ENT specialist should suspect Munchausen syndrome in patients with strange and long-lasting symptoms, so as to avoid misdiagnosis and unnecessary harmful treatments that waste time and resources in the free healthcare sector.

Patients with factitious disorders are often treated by multiple clinicians. Diagnosis requires exclusion of unusual presentation of common disorders and psychiatric evaluation to exclude borderline personality, conversion, delusional and somatic symptom disorders.[1,3,4] The management requires a multidisciplinary team: physicians and surgeons to provide acute emergency care,[10,11] psychiatrists to evaluate the mental health and support from family and social workers.[1] All members of the patient’s multidisciplinary team should be informed about the diagnosis of a factitious disorder and the treatment plan. The patient should be monitored for the risk of self-injury or suicide. The diagnosis should be discussed with the patient and the family members in a manner that minimizes humiliation to the patient. Specific therapy involves supportive psychotherapy or cognitive-behavioural therapy and engagement of patients in the treatment. Antipsychotics or antidepressants are not beneficial.[12] However, there is an absence of sufficient robust data on the effectiveness of any management technique for factitious disorders.[12] The prognosis for factitious disorders imposed on self is poor and requires long-term follow-up.

Munchausen syndrome poses diagnostic challenges especially in settings where access to specialist mental health practitioners are limited. The incidence of Munchausen syndrome in developing countries is not known and the patients are often misdiagnosed and do not receive appropriate care.[2,9] This is particularly concerning when access to mental health care is limited in many developing countries. Bhutan, a country in the eastern Himalayas, is one such example where there are only 376 doctors among which only three are psychiatrists.[13] As the forms of Munchausen syndrome change over time, such as a case of factitious movement disorder with a forged gene mutation testing reported in 2017[14] and the symptoms of dry cough, sore throat and anosmia mimicked during the COVID-19 pandemic,[15] this report is a timely reminder for the need to improve access to mental health professionals and cross-disciplinary collaboration in patient care.

Conclusion

Factitious disorders and Munchausen syndrome should be considered in patients with characteristic history and discrepancies with physical examination and investigative findings. Early investigation and confirmation of possible factitious disorder reduces harm to the patient and saves health resources.