Foreign Accent Syndrome after Retrosigmoid Craniotomy for Vestibular Schwannoma—A Case Report

Introduction: Foreign accent syndrome (FAS) is a rare speech disorder with a variety of reported etiologies. Diagnostic criteria of neurologic FAS include (1) the accent is considered by the patient, acquaintances, and physician to sound foreign; (2) the accent is different from speech prior to cerebral insult; (3) it is related to CNS damage and is not psychogenic; and (4) the patient does not have a history indicating a reason for having an accent. Most individuals have accompanying left cerebral hemisphere lesions or have a pathologic disruption between cerebral and cerebellar speech centers. Individuals who experience cerebrovascular accidents are particularly susceptible to FAS. It has been reported that problems with articulation and comprehension can eventually lead to FAS. Psychogenic FAS is sometimes considered a subcategory of conversion disorder and has common comorbid psychiatric conditions including depression, bipolar disorder, and schizophrenia and can present with additional conversion disorders. FAS can also present independent of other comorbid conditions.

Case Description: The patient is a 41-year-old female from the Midwest United States with no prior speech abnormalities, impediments, localizing dialect, or psychiatric history. Her past medical history includes migraine with aura, suspected trigeminal neuralgia, central sleep apnea, and a BMI of 65. She initially presented with headaches, facial pain, dizziness, and right-sided hearing loss and tinnitus. MRI revealed a right vestibular schwannoma. She underwent a right retrosigmoid craniotomy at an outside hospital with partial tumor resection. Intraoperatively, she experienced cerebral edema requiring partial resection of the right cerebellar hemisphere to permit closure of the craniotomy. When she awoke from surgery, she had developed an Irish accent. She also lost all residual ipsilateral hearing post-operatively. Voice abnormalities were not a part of her initial presentation. The accent has persisted without interruptions and remains present 7 years after surgery. She required a ventriculoperitoneal shunt post-operatively. She is otherwise neurologically intact and does not have dysphagia, scanning speech, stuttering, odynophagia, or difficulty with phonation or pronunciation. Her vocabulary is unchanged, and she has does not have expressive, conductive, or mixed aphasia. Apart from her persistent Irish accent, her speech is unchanged from prior to her surgical resection. She has never experienced these symptoms before.

Discussion: The patient has a clear history of a cerebral insult, and her cerebellar involvement is consistent with prior reported cases of FAS. Her partial cerebellar resection appears to have impacted her cerebral speech processing. The unique aspect of her presentation is the absence of any other speech abnormalities. Additionally, her only apparent auditory processing abnormality is her unilateral profound hearing loss status post vestibular schwannoma resection. Her Irish accent after a neurosurgical tumor resection supports a diagnosis of neurologic FAS and her case is made all the more interesting by her lack of additional speech abnormalities.

Publication History

Article published online:
15 February 2022