Abstracts
Migraine is a complex disease that includes neurologic, gastrointestinal and autonomic symptoms, although headache is most common feature. In a portion of cases headache is preceded by focal neurologic symptoms termed auras. Auditory symptoms only rarely occur as part of an aura. We describe a patient whose 13-year migraine history that included the abnormal perception an oscillation of the intensity of ambient sounds (oscillucusis). During a migraine attack immediately after oscillucusis, the patient developed acute and permanent sudden deafness. Clinical and neurologic examinations revealed only profound hearing loss in her left ear. Audiometric testing confirmed the sensorineural nature of the hearing loss. The clinical aspects and physiopathology of auditory symptoms in this case and in patients with migraine is reviewed.
aura; headache; migraine; oscillucusis; sudden deafness
Migrânea é desordem complexa que inclui sintomas neurológicos, gastrointestinais e autonômicos, na qual a cefaléia é o achado predominante. Em uma parcela de pacientes o quadro álgico pode ser antecipado por sinais neurológicos focais conhecidos como aura. Descrevemos um paciente que iniciou sua historia de migrânea acompanhada por sensações de flutuações nos sons ambientes (oscillucusis) por treze anos. Durante uma crise de cefaléia imediatamente após a oscillucusia o paciente desenvolveu um quadro agudo e permanente de perda auditiva. Exames clínicos e neurológicos revelaram somente uma perda profunda da audição em ouvido esquerdo, cuja natureza sensorioneural foi confirmada por uma avaliação audiométrica. Os aspectos clinicos e fisiopatológicos dos sintomas auditivos encontrados neste caso e em pacientes com migrânea com aura são revisados e comentados.
aura; cefaléia; migrânea; oscilucusia; perda súbita de audição
Oscillucusis and sudden deafness in a migraine patient
Oscilucusia e perda auditiva em um paciente com migranea
Elcio Juliato PiovesanI, II; Pedro Andre KowacsI; Lineu Cesar WerneckI; Charles SiowII
ISetor de Cefaléia, Especialidade de Neurologia do Departamento de Clinica Médica do Hospital de Clinicas da Universidade Federal do Paraná (UFPR), Curitiba PR, Brazil
IIJefferson Headache Center, Thomas Jefferson University Hospital, Philadelphia,PA, USA
ABSTRACT
Migraine is a complex disease that includes neurologic, gastrointestinal and autonomic symptoms, although headache is most common feature. In a portion of cases headache is preceded by focal neurologic symptoms termed auras. Auditory symptoms only rarely occur as part of an aura. We describe a patient whose 13-year migraine history that included the abnormal perception an oscillation of the intensity of ambient sounds (oscillucusis). During a migraine attack immediately after oscillucusis, the patient developed acute and permanent sudden deafness. Clinical and neurologic examinations revealed only profound hearing loss in her left ear. Audiometric testing confirmed the sensorineural nature of the hearing loss. The clinical aspects and physiopathology of auditory symptoms in this case and in patients with migraine is reviewed.
Key words: aura, headache, migraine, oscillucusis, sudden deafness.
RESUMO
Migrânea é desordem complexa que inclui sintomas neurológicos, gastrointestinais e autonômicos, na qual a cefaléia é o achado predominante. Em uma parcela de pacientes o quadro álgico pode ser antecipado por sinais neurológicos focais conhecidos como aura. Descrevemos um paciente que iniciou sua historia de migrânea acompanhada por sensações de flutuações nos sons ambientes (oscillucusis) por treze anos. Durante uma crise de cefaléia imediatamente após a oscillucusia o paciente desenvolveu um quadro agudo e permanente de perda auditiva. Exames clínicos e neurológicos revelaram somente uma perda profunda da audição em ouvido esquerdo, cuja natureza sensorioneural foi confirmada por uma avaliação audiométrica. Os aspectos clinicos e fisiopatológicos dos sintomas auditivos encontrados neste caso e em pacientes com migrânea com aura são revisados e comentados.
Palavras-chave: aura, cefaléia, migrânea, oscilucusia, perda súbita de audição.
Migraine is one of the most commom neurologic diseases that occurs in the general population1. The combinations of symptoms, including headache, neurologic, gastrointestinal and autonomic features1, the focal neurological phenomena that precede or accompany an attack are known as aura. These neurological manifestations include visual, somato-sensory, olfactory and less frequently auditory symptoms.
The case of a migraine patient with auditory aura followed by hearing loss is revised, and the literature about auditory symptoms in migraine with aura patients are reviewed.
CASE
A 56-year-old woman started to present migraine episodes at age 40. An year later she perceived ambient sounds to be fluctuating in intensity (oscillucusis). This would last 50 minutes, and was followed by a unilateral (right or left side), throbbing headache associated with nausea and vomiting. These episodes lasted 18 hours, occurred at twice a month and were commonly triggered by stress. At age 53, she had the sudden onset of profound hearing loss in her left ear that was unassociated with vertigo or any other neurologic symptoms. This symptom started 30 minutes after the oscillucusis and was followed by a migraine attack that lasted one day. The profound hearing loss in her left ear remained unchanged throughout the remainder of her life.
The patient's medical history was negative for hypertension, coronary artery disease, stroke, recent viral infection, and hypercoagulation syndromes (polycythemia and macroglobulinemia). Her family history revealed that her mother and daughter had migraine headaches. The general and neurologic examinations were normal except for the profound left-sided hearing loss. Complete hematologic and metabolic work-ups, including thyroid function studies, were normal. MRI and CT of the brain were normal. Audiometric testing showed a severe sensorineural hearing loss in the left ear with discrete sensorineural hearing loss in the right ear. Vestibular function testing performed with rotation, in the dark, at multiple frequencies and peak velocities were normal. Auditory evoked potential of the brainstem was normal.
The patient was started on propranolol 40mg/day. This improved her headache, decreasing its frequency to one attack every three months. It also decreased the intensity and duration of her headaches. The migraine has been controlled with propranolol for three years; however the auditory disturbance remained unchanged.
DISCUSSION
Migraine is a common neurologic disorder characterized by attacks that consist of various combinations of headache and neurologic, gastrointestinal and autonomic symptoms1. The clinical spectrum of this disorder is abundant and some patients will develop focal neurological symptoms that precede or accompany an attack of headache. These symptoms, called aura, consist of visual, sensory, or motor phenomena and may involve language or brain stem disturbances1. Auditory symptoms are less common than vestibular symptoms2-4. Phonophobia is probably the most common auditory symptom associated with migraine, occurring at some time in more than two thirds of patients, usually in association with headache5. Auditory symptoms also include: auditory hallucinations6, oscillocusis7, tinnitus8, fluctuating low-frequency hearing loss9, and sudden deafness10-12.
Auditory hallucinations as aura in migraine patients (AHAM) without psychotic disease has been reported in different studies6,13. The auditory hallucinations include voices accompanying the migraine attacks6,13, the sense that ambient sounds were fluctuating in intensity (oscillucusis)7, and tinnitus, which can occur in many conditions other than ototoxicity, such as AHAM8. In AHAM we need to eliminate other etiology, such as autosomal dominant partial epilepsy14 and thalamic and pontine auditory hallucinosis15.
Sudden hearing loss is defined as a severe to profound loss of hearing occurring over minutes to hours that is sensorineural in origin16. Sudden unilateral deafness occurring in a young patient is usually due to viral infection of the cochlea17-19, but the most common causes of sudden unilateral deafness are cerebrovascular diseases or hypercoagulation syndromes, such as polycythemia and macroglobulinemia20-22. Viirre at al described the sudden hearing loss in 13 migraine patients11, all of whom showed a sudden onset of hearing loss and other neurological phenomena that could be attributed to vasospasm, including vertigo, amaurosis fugax, hemiplegia, facial pain, chest pain, and visual aura11. Acute hearing loss can also be secondary to endolymphatic hydrops diseases23-24. In this situation, the acute stimulation of one of the otolith organs from the hydrops will produce drop attacks that may lead to confusion with vertebrobasilar insufficiency25. Migraine patients have reported the abrupt onset of a profound hearing loss26-28. Although some gradual improvement may occur, they are often left with a severe, unilateral, or bilateral27, sensorineural hearing loss. Some patients with sudden hearing loss report a previous history of fluctuating hearing in the same ear, and many develop persistent tinnitus29.
Previous reports have observed that migraine can lead to permanent auditory and vestibular deficits11,26,30-31. It is common that migraine-related transitory neurological symptoms lasting more than four and less than 60 minutes can be considered to be aura. The most common migraine aura is visual, and metabolic studies of the brain suggest a primary neuronal basis with secondary vascular changes, such as vasospasm or vasodilatation depending on the phase of migraine32-33. In retinal migraine this vasospasm may result in blindness34-35, illustrating vasospasm to have occurred at some time during the migraine attack12, 21,36.
The pathophysiology of auditory symptoms is probably related with vasospasm of small arterioles within the cochlea and labyrinth, much as retinal migraine may involve only a subset of arterioles in the retina11,12,22,37. However controversy exists regarding its role in the production of the symptoms29,37. Another way to explain the heterogeneity of inherited migraine syndromes is to postulate a group of defects in genes that code for a family of protein38. These proteins build the ion channels that, in migraine, produce an abnormal voltage-gated calcium-channels. The ion channels in the inner ear are critical for maintaining the potassium-rich endolymph and neuronal excitability. A defective ion channel shared by brain and inner ear could lead to reversible hair cell depolarization and auditory and vestibular symptoms29.
In our case the presence of oscillucusis before the headache phase of migraine suggests an auditory migraine aura. The etiology of this symptom could be related to transient vascular changes in the choclea during migraine attacks. Our patient has had an association between migraine and auditory aura for thirteen years, when she developed sudden and permanent deafness. Although the etiology of these symptoms remains to be determined, we believe that both oscillucusis and sudden and permanent deafness in migraine patients reflect localized aura phenomena and cochlear ischaemia, respectively.
Acknowledgements - The authors would like to thank to Jia Luo MD, Michael Oshinsky PHD, from the Headache Center Research Laboratory, Department of Neurology and Lynne Kaiser and Linda Algarin Kelly from the Jefferson Headache Center, Thomas Jefferson University Hospital, for technical support and suggestions.
Received 20 February 2003
Received in final form 6 May 2003
Accepted 29 May 2003
Dr. Elcio Juliato Piovesan - Rua Joinville 2526 - 83020-000 São José dos Pinhais PR - Brasil. E-mail: Piovesan@avalon.sul.com.br
- 1. Silberstein SD, Saper JR, Freitag FG. Migraine. Diagnosis and treatment. In Silberstein SD, Lipton RB, Dalessio DJ (EDS). Wolffs headache and other head pain. 7.Ed. Philadelphia: Ortho-McNeil 2001:121-237.
- 2. Kuritzky A, Ziegler DK, Hassanein R. Vertigo, motion sickness and migraine. Headache 1981;21:227-231.
- 3. Kayan A, Hood JD. Neuro-otological manifestations of migraine. Brain 1984;107:1123-1142.
- 4. Harker LA, Rassekh C. Migraine equivalent as a cause of episodic vertigo. Laryngoscope 1988;98:160-164.
- 5. Harker LA. Migraine-associated vertigo. In: Baloh RW, Halmagyi GM, (eds). Disorders of the Vestibular System. New York: Oxford Univ Press; 1996:407-417.
- 6. Rubin D, McAbee GN, Feldman-Winter LB. Auditory hallucinations associated with migraine. Headache 2002;42:646-648.
- 7. Whitman BW, Lipton RB. Oscillucusis: an unusual auditory aura in migraine. Headache 1995;35:428-429.
- 8. Seidman MD, Jacobson GP. Update on tinnitus. Otolaryngol Clin N Am 1996;29:455-465.
- 9. Parker W. Migraine and the vestibular system in adults. Am J Otol. 1991;12:25-34.
- 10. Olsson JE. Neurotologic findings in basilar migraine. Laryngoscope 1991;101(Suppl 52):1-41.
- 11. Viirre ES, Baloh RW. Migraine as a cause of sudden hearing loss. Headache 1996;36:24-28.
- 12. Lee H, Lopez I, Ishiyama A, Baloh RW. Can migraine damage the inner ear? Arch Neurol 2000;57:1631-1634.
- 13. Schreier HA. Auditory hallucinations in nonpsychotic children with affective syndromes and migraines: report of 13 cases. J Child Neurol 1998;13:377-382.
- 14. Winawer MR, Martinelli Boneschi F, Barker-Cummings C, et al. Four new families with autosomal dominant partial epilepsy with auditory features: clinical description and linkage to chromosome 10q24. Epilepsia 2002;43:60-67.
- 15. Louis E, Dupont S, Chochon F, Crozier S, Baulac M, Pierrot-Deseilligny C. Peduncular hallucinosis: implication of the thalamic and pontine structures. Rev Neurol (Paris) 2001;157:551-552.
- 16. Nomura Y. Diagnostic criteria for sudden deafness, mumps deafness and perilymphatic fistula. Acta Otolaryngol 1988(Suppl);456:7-8.
- 17. Schuknecht HF, Kimura R, Naufal PM. The pathology of sudden deafness. Acta Otolaryngol Stockh 1973;76:591-598.
- 18. Schuknecht HF, Donovan ED. The pathology of idiopathic sudden sensorineural hearing loss. Arch Otorhinolaryngol 1986;243:1-15.
- 19. Khetarpal U, Nadol JB, Glynn RJ. Idiophatic sudden sensorineural hearing loss and postnatal viral labyrintitits: a statistical comparison of temporal bone findings. Ann Otol Rhinol Laryngol 1990;99:969-976.
- 20. Amarenco P, Rosengart A, DeWitt LD, Pessin MS, Caplan LR. Anterior inferior cerebellar artery territory infarcts. Mechanisms and clinical features. Arch Neurol 1993;50:154-161.
- 21. Andrews JC, Hoover LA, Lee RS, Honrubia V. Vertigo in the hyperviscosity syndrome. Otolaryngol Head Neck Surg 1988;98:144-149.
- 22. Ruben RJ, Distenfeld A, Barg P, Carr R. Sudden sequential deafness as the presenting symptom of macroglobulinemia. JAMA 1969;209:1364-1365.
- 23. Schuknecht HF, Suzuka Y, Zimmermann C. Delayed endolymphatic hydrops and its relationship to Meniere's disease. Ann Otol Rhinol Laryngol 1990;99:843-853.
- 24. Harris JP, Aframian D. Role of autoimmunity in contralateral delayed endolymphatic hydrops. Am J Otol 1994;15:710-716.
- 25. Baloh RW, Jacobson K, Winder T. Drop attacks with Meniere's syndrome. Ann Neurol 1990;28:384-387.
- 26. Lipkin AF, Jenkins HA, Coker NJ. Migraine and sudden sensorineural hearing loss. Arch Otolaryngol Head Neck Surg 1987;113:325-326.
- 27. Lee H, Whitman GT, Lim JG, et al. Hearing symptoms in migrainous infarction. Arch Neurol 2003;60:113-116.
- 28. Caplan LR. Migraine and vertebrobasilar ischemia. Neurology 1991;41:55-61.
- 29. Baloh RW. Neurotology of migraine. Headache 1997;37:615-621.
- 30. Cutrer FM, Baloh RW. Migraine-associated dizziness. Headache 1992;32:300-304.
- 31. Harker LA, Rassekh C. Migraine equivalent as a cause of vertigo. Laryngoscope 1988;98:160-164.
- 32. Kaube H, Knight YE, Storer RJ, Hoskin KL, May A, Goadsby PJ. Vasodilator agents and supracollicular transection fail to inhibit cortical spreading depression in the cat. Cephalalgia 1999;19:592-597.
- 33. Aurora SK, Welch KM. Migraine: imaging the aura. Curr Opin Neurol 2000;13:273-276.
- 34. Glenn AM, Shaw PJ, Howe JW, Bates D. Complicated migraine resulting in blindness due to bilateral retinal infarction. Br J Ophthalmol 1992;76:189-190.
- 35. Hupp SL, Kline LB, Corbett JJ. Visual disturbances of migraine. Surv Ophthalmol 1989;33:221-236.
- 36. Tippin J, Corbett JJ, Kerber RE, Schroeder E, Thompson HS. Amaurosis fugax and ocular infarction in adolescents and young adults. Ann Neurol 1989;26:69-77.
- 37. Farkkila M. Pathophysiology of migraine. Ann Med 1994;26:7-8.
- 38. Baloh RW, Foster CA, Yue Q, Nelson SF. Familial migraine with vertigo and essential tremor. Neurology 1996;46:458-460.
Publication Dates
-
Publication in this collection
12 Nov 2003 -
Date of issue
Sept 2003
History
-
Accepted
29 May 2003 -
Received
20 Feb 2003