The man who fell in love with the sound of Spitfires – here’s what this unusual symptom can teach us about dementia

When people hear the word dementia, they often think of someone who has problems with memory. While memory is often affected in dementia, this is not always the case. There are many different types of dementia – and each can produce a wide range of symptoms.

A recent case study has even described a 68-year-old man with a rare form of dementia that caused him to develop a fascination with a very specific type of noise. As this type of dementia has only been recently recognised by medical experts, this finding suggests that changes in preferences for sounds may be a key feature of the syndrome.

Dementia is an umbrella term to describe cognitive (thinking) problems that are severe enough to affect everyday life. There are many types of dementia – such as Alzheimer’s disease, which is the most common form. It is characterised by memory loss and other cognitive changes.

Another subtype is frontotemporal dementia, which tends to affect people before age 65.

As the name suggests, frontotemporal dementia affects brain regions in the frontal and temporal lobes, which sit behind the forehead and above the ears. These areas of the brain are involved in a wide range of functions – including personality, behaviour, problem solving, planning, language, processing and understanding sounds. This form of dementia mainly affects behaviour or language abilities.

There are three main variants of frontotemporal dementia, each of which have differing symptoms: the behavioural variant (affecting behaviour and personality), the non-fluent variant (affecting speech production) and the semantic variant (affecting knowledge and understanding speech).

But some researchers believe there’s a fourth variant of frontotemporal dementia, as well. Evidence for this fourth variant was described in a case study I helped conduct.

A 68-year-old man, who we called “CP”, developed an unusual love for Spitfire engine noises. CP’s wife first noticed this strange behaviour about two years before he was diagnosed with dementia.

CP lived near an airfield, and veteran aircraft would frequently fly over his home. He would drop whatever he was doing and run outside, waving at the planes and crying tears of joy whenever he heard them. He had never reacted this way before the onset of his condition.

His love for engine noises was very specific to this type of plane. He did not react the same way to the sound of other planes, nor show a general interest in aircraft or vehicles. He also found birdsong and people with high-pitched voices irritating. He even became very particular about music, disliking covers and preferring originals.

A few years before his sudden love for Spitfire noises began, CP became moody and short-tempered. He became increasingly cold and apathetic towards others and lacked impulse control and awareness of socially acceptable behaviour. For instance, he was indifferent to a death in the family and frequently interrupted other people when they were speaking – things he would have never done before his disease.

He also lost understanding of humour, developed a sweet tooth and became fixated on playing chess and doing word searches. He sometimes failed to recognise the faces of acquaintances but did not have problems recognising people’s voices over the telephone. CP did not show any problems with remembering previous events or language.

About five years after symptoms emerged, CP was diagnosed with behavioural variant frontotemporal dementia. But we believe CP had a newer, fourth variant – sometimes referred to as the “right temporal variant”.

This variant was given its name because most of the tissue loss occurs in the right temporal lobe of the brain. This brain area is mainly involved in understanding concepts and deriving meaning from nonverbal information, such as social cues. Scans of CP’s brain showed that large portions of this region were missing.

The right temporal variant of frontotemporal dementia also appears to cause a mix of symptoms typically seen in both the behavioural and semantic variants. However, there’s still debate within the research community over how to define it.

Learning about dementia

There’s a lot that can be learned from CP.

First, his story helps spread awareness of frontotemporal dementia. Lack of awareness is a major issue – even among doctors, as it’s commonly misdiagnosed as other psychiatric illnesses or Alzheimer’s disease.

CP’s story also helps to clarify the right temporal variant of frontotemporal dementia. His symptoms suggest that the development of new fixations may be a defining feature of the syndrome.

CP’s case is also an example of how dementia can cause changes in how people process sounds.

A link between hearing impairment and dementia is already well established. However, the nature of this relationship is unclear.

Although it has been widely claimed in the media that hearing loss causes dementia, it might also be the other way around – that dementia causes hearing changes. CP’s story provides evidence of this, as his abnormal love for specific noises only occurred after disease onset.

Alzheimer’s disease research also supports the idea of dementia causing hearing changes. For instance, impairments in auditory scene analysis – the ability to separate overlapping sounds, such as listening to one speaker among background noise – has emerged as a common symptom.

CP’s story also demonstrates how dementia can change what people find pleasurable as well as their emotions. Intense obsessions, aversions and changes in preferences (such as suddenly loving or hating certain foods, music or colours) have been widely reported in frontotemporal dementia.

I had the pleasure of meeting CP and his wife and learning about their dementia journey first-hand. CP’s story illustrates how important it is to recognise the variety of symptoms in dementia. This will in turn help lead to earlier diagnosis and the development of tailored interventions.

Lucy Core was supported by the UCL Research Excellence Scholarship while the case study was conducted and received funding from the Royal National Institute of the Deaf while preparing this article.